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Schwannomas are benign tumours derived from Schwann cells and are extremely rare in the biliary tract. We present the case of a 62-year-old patient with a common bile duct schwannoma that resembled a cholangiocarcinoma. We also review all 17 previously published cases of schwannoma of the biliary tract and discuss the challenges of preoperative diagnosis. KEYWORDSBile ducts -Cholangiocarcinoma -Differential diagnosis -Obstructive jaundice -Schwann cells Although cholangiocarcinoma is the most common biliary neoplasm causing obstructive jaundice, other malignancies, such as lymphoma or neuroendocrine tumours can be implicated. Currently, only 17 cases of biliary schwannoma, a benign tumour of the bile ducts, have been reported in the literature. We present the case of a patient with a biliary schwannoma that resembled a cholangiocarcinoma and caused jaundice. Case reportA 62-year-old man presented with obstructive jaundice and abdominal pain. His past medical history included moderate aortic insufficiency, hepatic steatosis and cholecystectomy. Laboratory tests revealed hyperbilirrubinemia (7.56 mg/dL, normal range 0.2-1.3 mg/dL), raised levels of aspartate transaminase 618 U/L (normal range 4-38 U/L), alanine transaminase (924 U/L, normal range 5-41 U/L), gamma-glutamyl transferase (1034 U/L, normal range 7-32 U/L) and alkaline phosphatase (189 U/L, normal range 4-129 U/L), and an international normalised ratio of 1.05 (normal range <1.3).Abdominal ultrasound showed mild dilation of the intrahepatic biliary tree, with normal extrahepatic biliary ducts. Magnetic resonance cholangiopancreatography (MRCP) revealed both intrahepatic and extrahepatic bile duct dilation secondary to a focal stenosis near the confluence of the right and left hepatic ducts. Subsequent endoscopic retrograde cholangiopancreatography confirmed the stenosis with proximal dilation (Fig 1). Biliary brushings were benign on cytology and a stent was placed. Computed tomography scan revealed no further information as to extent of the disease. Tumour marker levels were 0.68 ng/mL (normal range 0-3 ng/mL) for carcinoembryonic antigen, 1.85 ng/mL (normal range <10 ng/mL) for alpha-fetoprotein and 30.53 U/mL (normal range <30 U/mL) for carbohydrate antigen 19-9.The patient was discussed at the multidisciplinary team meeting and a presumptive diagnosis of cholangiocarcinoma was reached. Management options were discussed with the patient, and it was decided to proceed to operative intervention.At the operation, the biliary tree appeared unremarkable, with no palpable mases. The liver was normal and no ONLINE CASE REPORT Ann R Coll Surg Engl 2016; 98: e143-e146

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