The clinical manifestation of a choledochal cyst is diverse and can mimic common diseases like gallstones, cholecystitis or pancreatitis. Initial diagnosis is often suspected after ultrasound of the biliary tract and confirmed with more specific studies as magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography. The risk of malignant transformation is well documented and the mainstay of therapy is excision of the choledochal cysts along with the gallbladder. Choledochal cysts are a rare phenomenon and only three cases were diagnosed in Iceland in the years 2000-2010. The following is a description of one of these cases along with an overview of the literature.