We experienced a case of endocrine cell carcinoma of the gallbladder with anomalous pancreaticobiliary ductal junction. It is well known that anomalous pancreaticobiliary ductal junction has potentiality to cause gallbladder cancer and that most of the cases are papillary adenocarcinoma or well-differentiated adenocarcinoma. The direct relationship between endocrine cell carcinoma of the gallbladder and anomalous pancreaticobiliary ductal junction has not been elucidated.