Anomalies of the liver are infrequent. Agenesis of the right hepatic lobe is an uncommon, usually asymptomatic, entity that is diagnosed through new imaging techniques or during surgery. We present the case of a 51-year-old woman with right hepatic lobe agenesis who underwent technically difficult cholecystectomy for symptomatic gallstones. We discuss the diagnosis and technical difficulties that can be encountered during cholecystectomy when there is right hepatic lobe agenesis.