In the past, congenital cystic dilatation of the intrahepatic bile ducts (Caroli’s disease) was thought to be quite rare. Dayton et al in 1983 were able to collect a total of 142 cases from the world literature including 4 new cases in their own series. However, Mercadier in 1987 claimed that only 40 to 50 authentic cases have been reported in the world literature. Since 1980 reports of this disease have been found in Chinese literature. With the advent and development of various imaging diagnostic technics, as well as more awareness of the disease, its incidence is gradually growing, more than 150 cases have been reported in Chinese literature recently. The author of this article was able to collect 40 cases of the disease in the period of 1972-1989, which was the larg In the past, congenital cystic dilatation of the intrahepatic bile ducts (Caroli’s disease) was thought to be quite rare. Dayton et al in 1983 were able to collect a total of 142 cases from the world literature including 4 new cases in their own series. However, Mercadier in 1987 claimed that only 40 to 50 authentic cases have been reported in the world literature. Since 1980 reports of this disease have been found in Chinese literature. With the advent and development of various imaging diagnostic technics, as well as more awareness the author of this article was able to collect 40 cases of the disease in the period of 1972-1989, which was the larg