报道１７例先天性胆总管囊肿病人，最常见的临床表现为腹痛１００％，间歇性黄疸３５５％，腹块１１８％，无１例有上述典型三联症诊断主要依靠Ｂ超、ＰＴＣ及ＥＲＣＰ手术方式主要为：囊肿部份切除空肠Ｒｏｕｘ－Ｙ吻合，囊肿切除空肠Ｒｏｕｘ－Ｙ吻合，前者并发症达４０％，癌变率占１０％；后者近远期疗效满意结果表明：先天性胆总管囊肿的手术治疗应尽可能切除胆总管囊肿；困难者可大部切除，但应避免残留囊腔过大，保证内引流口在囊肿最低位，减少胆汁潴留及反复感染，才能降低结石和癌变的发生 Reported 17 cases of patients with congenital choledochal cyst, the most common clinical manifestations of abdominal pain was 100%, intermittent jaundice 35  5%, 11.8% of the abdominal mass, no one case of the above-mentioned typical triad ﹑ diagnosis depends mainly on B-ultrasound, PTC and ERCP  surgical methods are mainly: partial removal of jejunum Roux-Y cyst anastomosis, cyst resection of jejunum Roux-Y anastomosis, the former complications of up to 40%, cancer rate of 10%; the latter short-term efficacy of satisfactory results  : Surgical treatment of congenital choledochal cyst should be removed as much as possible choledochal cyst; difficult to remove the majority of patients, but should avoid residual cysts is too large to ensure that the drainage outlet in the lowest cyst, reduce bile retention and recurrent infection in order to Reduce the occurrence of stones and cancers