Agenesis of the gallbladder and cystic duct represents one of the rarest anomalies of the biliary system, with a reported incidence of 0.007% to 0.027%. Almost half of the patients develop common duct stones and 23% of them manifest signs and symptoms that mimic biliary colic. We present the case of a woman presenting with symptoms of biliary colic. Based on the clinical findings and after abdominal ultrasonography, which showed hyperechoic material in the gallbladder fossa, a laparoscopic cholecystectomy was planned. Laparoscopy failed to reveal either gallbladder or cystic duct. The procedure was continued to further search for ectopic sites of gallbladder. A condition of gallbladder agenesis was hypothesized and the procedure was aborted without dissection of hepatic pedicle or conversion to laparotomy. Agenesis of gallbladder and cystic duct was confirmed via pos-operative magnetic resonance cholangiopancreatography. We report our experience with regard to the challenges associated with the diagnosis and management, and a brief review of the literature of this rare pathology.