Some studies have reported on laparoscopic excision for treating the choledochal cyst, yet there are no reports on laparoscopic surgery for treating type IV-A choledochal cysts that require a liver resection. In this paper, we report on a case of laparoscopic cyst excision combined with left hemihepatectomy and laparoscopy-assisted Roux-en-Y hepaticojejunostomy for treating a type IV-A choledochal cyst. A 51-year-old female was admitted with symptoms of jaundice and cholangitis. Percutaneous transhepatic biliary drainage (PTBD) was done preoperatively for controlling the cholangitis. The imaging studies revealed a type IV-A choledochal cyst with an associated stricture of the left main intrahepatic duct. After the resolution of the cholangitis, total laparoscopic cyst excision and left hemihepatectomy were performed by using the four-port technique, and then a Roux-en-Y hepaticojejunostomy was done by a laparoscopy-assisted method. The total operation time was 420 minutes. The estimated blood loss was 300 mL, and no perioperative transfusion was needed. The tubogram, which was performed through the PTBD on postoperative day 5, showed good patency of the bilioenteric anastomosis and no biliary leakage. The patient was discharged at postoperative day 7 without any complications. This case shows the feasibility of performing laparoscopic surgery for treating a type IV-A choledochal cyst that requires a liver resection. We believe that laparoscopic cyst excision with a liver resection can be one of the treatment options for selected patients with type IV-A choledochal cysts.