Agenesis of the gallbladder (AGB) without extrahepatic biliary atresia is a rare congenital disease. Ultrasound (US) examination can be misleading and reveal a contracted shrunken gallbladder when there is not any and the patient in most cases is taken to the OR for a standard cholecystectomy. We describe the case of a 54-year-old female with colicky right upper abdominal pain with nausea. US revealed a contracted scleroatrophic gallbladder and the patient was listed for laparoscopic cholecystectomy. At laparoscopy, despite careful search, the gallbladder was never visualised, and the suspicion of AGB was raised. An intra-operative cholangiography confirmed the hypothesis. The post-operative recovery was uneventful, and abdominal computed tomography scan failed to show the presence of gallbladder, therefore confirming the diagnosis of AGB. Lack of awareness of this condition among radiologists and surgeons is the main reason for unnecessary operations and potentially damages to the biliary tract.