Neurofi broma of the porta hepatis

Neurofi bromas are generally well-circumscribed tumors of the nervous system, often found in association with neurofi bromatosis 1 (NF1). They uncommonly present as a single sporadic form in the retroperitoneum and in the pancreas. We present a case of a 40-year-old man who presented with a 4- to 5-year history of right upper quadrant pain with associated nausea and vomiting. Medical imaging showed a 3.8 by 3.4-cm mass encasing the hepatic artery and the porta hepatis. Surgical resection was performed, with removal of the gallbladder. The mass was histologically diagnosed as a neurofi broma. At 1 year following his surgery, the patient is doing well.