RESUMO
Granular cell tumors (GCTs) are rare benign neoplasms that are most often located in the soft tissues of the extremities and chest wall. Malignant GCTs have also been reported. GCTs of the biliary tract are extremely rare, uncommon non-epithelial benign neoplasms that cause focal thickening of bile duct wall without mucosal invasion. They consist of polygonal cells with granular appearance and stain positive in S-100 protein, indicating a neural (Schwann cell) origin. We report our experience of a 57-year-old Caucasian woman who presented with obstructing jaundice due to a distal bile duct stricture highly suspicious of cholangiocarcinoma. A Whipple's procedure was successfully performed, and the final pathology revealed a benign GCT of the distal bile duct. Whipple's is an extremely radical procedure for such benign lesions and additional investigations, such as cytology sample, may result in a less aggressive approach as those tumors grow slowly and do not metastasize.