肝胆管囊腺癌(Biliary cystadenocarcinoma简称BCAC)属肝内罕见恶性肿瘤,迄今全世界报道不到30例。本文报告国内首例,并进行讨论。病例报告患者女性,58岁。1982年发现上腹有一鸡蛋大小肿物。无不适。近2年肿物渐增至儿头大小伴上腹隐痛,食后饱胀,于1986年4月入院。无其它肝病史及脏器先天性畸形史。体检:一般情况好。中上腹膨隆,剑突下可触及16×14cm固定之囊性肿物,表面光 Biliary cystadenocarcinoma (BCAC) is a rare intrahepatic malignancy, so far less than 30 cases reported worldwide. This article reports the first case in China and discusses it. Case report Female patient, 58 years old. In 1982 found that the size of an egg on the abdomen tumor. No discomfort In the past 2 years, the tumor gradually increased to the size of the abdomen accompanied by abdominal pain, fullness after eating, and was admitted to hospital in April 1986. No other history of liver disease and organ history of congenital malformations. Physical examination: the general situation is good. Abdominal bulge, xiphoid can reach 16 × 14cm fixed cystic mass, the surface light