RESUMO
Granular cell tumour (GCT) is a rare soft tissue neoplasm commonly encountered in the head and neck region, skin and subcutaneous tissue. GCT of the biliary system is most commonly reported in African-American females and usually presents as abdominal pain and obstructive jaundice. The neoplasm constitutes less than 10% of all benign tumours of the extra-hepatic biliary tree. No case of GCT involving the ampulla of Vater has been reported in the literature to date. We report a case of benign GCT involving the ampullary region in a 44-year-old Ethiopian male. Preoperative diagnosis was available on ERCP and deep biopsy. The patient was managed by debulking resection and biliary-enteric bypass and is symptom-free with no evidence of tumour progression after a follow-up of one year.