Schwannoma or neurilemmoma is an uncommon neoplasm, and it is derived from the peripheral nerve sheath. These tumors most frequently occur in the head, neck and flexor surfaces of the upper and lower extremities, while the porta hepatis and retroperitoneum are rarely implicated. We report here on a case of benign schwannoma in the porta hepatis with compression of the common bile duct, and this was successfully treated by surgical excision. The patient was a 63-year-old woman who had a slow growing upper abdominal mass for 3 years. The abdominal CT scan showed a 12 cm-sized cystic mass with a solid portion, and this was accompanied with intrahepatic bile duct dilatation. Abdominal exploration revealed a yellowish, cystic, encapsulated mass attached to the inferior vena cava, portal vein, the common bile duct and the liver. There has been no evidence of recurrence during 36 months of follow-up.