Most population-based series reporting on the coronary artery complications after Kawasaki disease (KD) originate from Japan. This study aimed to describe the complete series of KD patients from the province of Quebec in Canada, a predominantly Caucasian population. This retrospective case series was conducted by the Quebec Kawasaki Disease Registry, a multi-institutional collaboration reviewing 89.8 % of all KD cases identified by the Ministry of Health records of hospitalization for KD from the first recognized case in 1976 until 2008. This report describes the course of 38 patients (95 % Caucasians) with a diagnosis of giant coronary artery aneurysms, which represent 1.9 % of all reviewed cases and 26.2 % of those with a coronary aneurysm 5 mm or larger. The age at diagnosis was 5.52 ± 4.04 years, and the follow-up period was 9.26 ± 6.9 years. The KD diagnosis was retrospective at autopsy in two cases and via echocardiography in four cases. The overall freedom from coronary thrombi, coronary intervention, or death was respectively 63.9, 67.5, and 85.1 %. Five deaths occurred as follows: 21 days after onset of fever (2 cases), 1.8 months after onset of fever (1 case), 1 year after retrospectively presumed but previously undiagnosed KD (1 case), and 5.7 years after a KD diagnosis (1 case of sudden cardiac death). Percutaneous transluminal coronary revascularization was attempted in four cases (1 requiring cardiac transplantation), and two other cases underwent primary bypass graft surgery. Whereas this study investigated cases of KD with severe coronary sequelae in the Province of Quebec, larger collaborative studies should be conducted for further understanding of the disease in predominantly non-Asian populations.