Congenital agenesis of the right liver (ARL) is a rare condition that is generally asymptomatic. Congenital anomalies of the liver are generally diagnosed with current cross-sectional imaging modalities such as ultrasound, computed tomography, and magnetic resonance imaging (MRI) before surgery. However, discovery of a congenital anomaly of the liver only at time of surgery remains a possibility. Herein reported is the case of a 68-year-old woman undergoing laparoscopic cholecystectomy for symptomatic gallbladder stones as diagnosed on preoperative ultrasounds. Upon laparoscopic exploration of the upper abdomen, the right liver was not found; the gallbladder was located in the right subdiaphragmatic region posterior to the medial segment of the liver. A posterolateral interposition of the hepatic flexure of the colon was also found. Cholecystectomy was completed under laparoscopy. A postoperative MRI confirmed right liver agenesis. We discuss the technical difficulties of performing a laparoscopic cholecystectomy in the case of ARL and the advantages of a laparoscopic approach.