The case of a 57-year-old woman with a benign biliary stricture is described. Radiological examinations showed granularly elevated lesions in the common hepatic duct and severe stenosis at the hepatic hilum. Under a tentative diagnosis of hepatic hilar bile duct cancer, the patient underwent extended right hepatectomy with bile duct resection. However, pathological and immunohistochemical studies revealed a benign stricture with remarkable formation of lymph follicles with germinal centers, which could be termed "follicular cholangitis". This is the first case report of this uncommon presentation of inflammatory changes of the bile duct. Its etiology was unknown, and more investigation is encouraged to clarify its relationship to systemic disorders and malignancies. As difficulty remains in discriminating between benign and malignant strictures by nonsurgical modalities at present, surgical resection without complications provides a feasible approach to such a benign lesion.