BackgroundExtrahepatic cyst excision and Roux-en-Y hepaticojejunostomy is not satisfactory in many patients with complex Todani type IV-A choledochal cysts. AimsTo report the results of combined extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts. MethodsThe records of patients who received extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts from January 2002 to December 2011 were retrospectively reviewed, and surgical outcomes analysed. Results59 patients (30.5% males; mean age, 43.2±18.4 years) were included. Radical excision of cystically dilated bile ducts was achieved in 53 patients (89.8%). Bile leakage, delayed wound healing, and abdominal infection occurred in 5 (8.47%), 7 (11.86%), and 3 (5.08%) patients, respectively. Forty-nine patients (83.1%) were followed for an average of 42.6±15.3 months. During the follow-up, 6 patients (12.2%) experienced recurrent cholangitis. Long-term biliary function was excellent in 33 (67.4%), good in 9 (18.4%), fair in 5 (10.2%), and poor in 2 (4.1%) patients. ConclusionCombined extra-hepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy is effective for the treatment of complex Todani type IV-A choledochal cysts with substantial intrahepatic bile duct involvement and hilar bile duct stenosis.