A 68-year-old woman was admitted to the department of internal medicine in our hospital because of fever and vomiting. Since increases in biliary enzymes were noted and endoscopic retrograde cholangiopancreatography (ERCP) revealed multiple occupying lesions in the common bile duct, the patient was diagnosed as having multiple tumor in the common bile duct associating with cholangitis. The patient was referred to the department and operated on. Resected specimen revealed multiple cysts in the common bile duct, which were biliary microhamartoma histopathologically. This disease usually arises in the liver and most patients with the disease are asymptomatic. This case which presented cholangitis like symptoms and arised in the common bile duct appears to be rare. In addition, it is believed that the disease is associated with adult type multiple cystic kidney in a high rate, and this patient was also associated with the multiple cystic kidney. It is suggested that the multiple tumors in the common bile duct in this case might be a lesion of general multiple cystic disease.