Benign ganglioneuroma (GN) contains mature autonomic ganglion cells including satellite cells and long axonal processes as well as Schwann cells [
1‐
3]. Those tumours originate from neural crest-derived cells which form the adrenal medulla and the sympathetic nervous system during embryonic development. Hence, they mainly arise in the mediastinum, retroperitoneum and pelvis where sympathetic ganglia are localized. Alike neuroblastoma and ganglioneuroblastoma, GN is supposed to represent the most mature neuroblastic tumour since ganglion cells that originate from neuroblasts are present in all of these tumours. Maturation of a neuroblastoma into a ganglioneuroma or ganglioneuroblastoma has been commonly described in the literature [
1‐
3].
GN occurs in children and young adults, and median age at diagnosis is 7 years [
4‐
7]. Distribution between males and females vary from a preference of the female gender [
5] to no gender difference [
7]. A higher frequency is reported in patients with multiple endocrine neoplasia type II and neurofibromatosis type 1. Due to their slow growth, most GNs are found incidentally. Most patients experience long-term disease-free survival even after incomplete resection [
5,
7]. Malignant transformation is rare [
8]. Since only a minority of GN are functional, distinct clinical symptoms are missing [
4,
5]. Rarely, these tumours may produce hormones, such as catecholamines, vasointestinal peptides and androgens [
7]. Radiographically, GN usually present as well-defined solid masses and do not contain cysts as opposed to neuroblastoma and ganglioneuroblastoma [
6,
9]. Non-enhancement or slight enhancement in arterial phase and progressive mild enhancement in delayed phase is seen [
6,
10]. At MR imaging, GN has low signal intensity on T1-weighted images and high signal intensity on T2 weighted images [
6]. Distinct radiological features are lacking. Therefore, diagnosis can be challenging and is more precisely achieved by histological examination after resection. An imaging-guided core needle biopsy can be a reasonable approach to enable a reliable diagnosis before major surgery.