Neurofibroma is an autosomal dominant genetic disease which is originated from the abnormal differentiation of neural crest cells and would cause system damage.NF can occur at most organs in the body,while it is rarely seen in the common bile duct.In February 2007,1 old patients with NF was admitted to the Lishui Central Hospital of Zhejiang Province.The results of preoperative magnetic resonance cholangiopancreatography (MRCP) revealed that the common bile duct was slightly dilated,and the diameter of the common bile duct was 1.2 cm.A nodular short T2 signal lesion was detected at the middle part of the common bile duct with the size of 1.4 cm × 1.6 cm.The results of intraoperative rapid frozen section pathological examination showed that the lesion was cholangiocarcinoma,then cholangiocarcinoma resection + biliojejunal Roux-en-Y anastomosis was applied to the patient.Postoperative pathological examination confirmed that the lesion was neurofibroma.The patient was followed up for 7 years and died of pulmonary infection in June 2014.The clinical presentation of neurofibroma of the common bile duct is untypical.Space-occupying lesions detected in the patients with history of bile duct injury or operation should be considered to be diagnosed as neurofibroma.Special attention should be paid to the accuracy of the results of rapid frozen section pathological examination in order to avoid missdiagnosis.