Choledochal cysts are uncommon congenital anomalies of the biliary tree. We report a 25-year-old female who presented with palpable abdominal lump, jaundice and fever. She was diagnosed with a huge, type IVA choledochal cyst with enormous asymmetric cystic dilatation of extrahepatic segment of left hepatic duct with the help of ultrasound and magnetic resonance cholangiopancreatography (MRCP). The patient experienced complete recovery after total resection of the entire extrahepatic cyst and Roux-en-Y hepaticojejunostomy at the level of the hilum. We discuss the clinical features, imaging characteristics and treatment of this giant Todani's type IVA choledochal cyst.