Background : Choledochal cyst is a rare congenital dilatation of the biliary tree and is uncommonly presented in adults. The size of a choledochal cyst varies but rarely exceeds 9 cm.

Methods : Reporting a rare case of large Todani type IVA choledochal cyst in an adult patient

Results : A 25-years-old female came in with worsening right upper quadrant abdominal pain accompanied by jaundice and fever. She had no known history of jaundice before and started noticing a yellowish discoloration of her eyes and skin over the past six months. She also experienced a stabbing-like abdominal pain that lasted for hours at a time in her right upper quadrant. The MRCP demonstrated a severe biliary obstruction due to multiple intra and extrahepatic choledochal cysts with the largest size of 7,6x7cm and accompanying gallbladder hydrops. After initial management of cholangitis, we performed a left lateral segmentectomy of the liver and excision of the external bile duct with a hepaticojejunostomy reconstruction. The patient recovered well after surgery. Upon outpatient follow-up, there was no evidence of the previous symptoms, and the patient had gone back to work and everyday daily life. We planned for a follow-up MRCP scheduled three months after surgery.

Conclusions : A successful management of a rare large todani type IV-A choledochal cyst in an adult.