RESUMEN
Biliary Schwannoma is an uncommon tumor in the digestive system and extremely rare in the bile duct. We report 48 year-old male with an incidentally found in the CT, a tumor in the hepatic hilum (3.2 cm), abutting the first portion of the duodenum, without common bile duct obstruction or lymphadenopathy. The patient was asymptomatic and had normal tumor markers. The endoscopic ultrasound (EUS) demonstrated contact between the tumor, common bile duct and right hepatic artery and the biopsy suggested of mesenchymal tumor. Tumor board recommended excision of the tumor, due to the risk of malignancy and biliary obstruction. The tumor was resected through laparoscopic approach, with a Roux-en-Y hepatico-jejunostomy. The histological report showed a biliary Schwannoma (positivity for S100 and focal GFAP), which infiltrated the external wall of the bile duct, without affecting the cystic duct, surgical margins were free of neoplasia.