Ciliated foregut cysts are rare anomalies due to aberrant embryological development. Only a small number of gallbladder ciliated foregut cysts have been reported. We report the case of a 29-year-old woman presenting with epigastric pain associated with diarrhoea and vomiting, who was found to have raised serum bilirubin levels and abnormal liver function tests. Following a diagnostic pathway including abdominal ultrasound, magnetic resonance cholangiopancreatography and endoscopic ultrasound the gallbladder cyst was provisionally diagnosed to be a cyst arising from the cystic duct or a duplicated gallbladder. A laparoscopic cholecystectomy was carried out and histopathology identified a ciliated foregut gallbladder cyst. The postoperative course was uneventful. In this report we offer what we believe to be an optimal diagnostic pathway and therapeutic strategy for this rare congenital cyst.