CN type II, unlike type I, is a potentially benign disease. Patients with this condition usually have serum bilirubin levels ranging between 10 and 20 mg/dL (175 and 350 μmol/l) and seldom develop kernicterus, although neurological dysfunction may occur if hyperbilirubinemia is exacerbated by fasting, drugs or infectious diseases [
11]. There have been several published reports of increased incidence of gallstones in patients with UDP-GT mutations [
2‐
5,
12]. In a series with 20 patients with CN type I followed over a 16-year period, half developed cholelithiasis and required elective cholecystectomy [
12]. Gallstones were detected on cholecystography in 4 of 53 patients with Gilbert’s disease by Foulk et al. [
3] and required cholecystectomy in 2 of 55 patients studied by Powell et al. [
2] In a series of cholecystectomy patients, Gilbert’s disease was reported in 7.5 % of male patients [
4]. Several explanations for this occurrence have been suggested. One theory relates to the composition of bile which is markedly abnormal in patients with UDP-GT mutations [
9,
13]. Due to the inability to adequately conjugate bilirubin, the bile of patients with UDP-GT mutations exhibits abnormal amounts of bilirubin mono and diglucuronides. These compounds are more prone to deconjugation by bacterial glucuronidases. Unconjugated bilirubin combines with bile calcium, leading to the formation of pigmented gallstones [
9,
13]. This however, may prove to be insufficient as a physiopathological explanation, since most reports of gallstones in patients with UDP-GT mutations describe an additional factor such as cystic fibrosis, sickle cell disease or spherocytosis [
6‐
8]. Nevertheless, despite an elevated risk for gallstone disease, we have only found one report of cholangitis in a patient with CN type II [
10]. Differentiation between Gilbert’s disease and CN type I and II is important since treatment and prognosis are different between these entities. Although the diagnosis is usually made on clinical grounds, response to phenobarbital (decrease by at least 25 % in CN type II), bile analysis, measurement of UDP-GT on liver tissue and genetic testing can be used to confirm the diagnosis [
14‐
17]. Interestingly, patients with atypical presentations preventing a clear differentiation between type I and II have been described, raising awareness for possible phenotypes with incomplete penetration [
18,
19]. Although generally unnecessary, treatment options for patients with CN type II include phototherapy [
1], plasmapheresis [
20], enzyme induction with phenobarbital [
4] and liver transplantation [
1,
21]. Phototherapy effectively decreased unconjugated bilirubin by converting it to a water-soluble photoisomer that can be excreted in the bile. This treatment is only temporary as skin thickness and body surface area increase with age, reducing its efficiency. Additionally, phototherapy represents a heavy burden for the patient, requiring 10 to 12 h/day of treatment. The average life expectancy for patients having type I syndrome is around 30 years [
21]. Even though the prognosis of CN type II is usually good and is compatible with a normal life span, repeated episodes of kernicterus may culminate in permanent neurological damage [
22,
23]. The present case seemed of great interest due to the rarity of the situation, and also because of the advanced age of presentation, with the patient only presenting this frequent complication at this old age. Aside from the episode of acute gallstone pancreatitis, 7 years earlier, no other significant complications of the prolonged hyperbilirubinemia were seen in her lifetime.