Agenesis of the right lobe of the liver is a rare congenital anomaly and only 29 cases have been reported in the literature. We encountered a patient with this anomaly diagnosed by ultrasonography, Computed tomography (CT), hepatobiliary scintigraphy, endoscopic retrograde cholangiopancreatography (ERCP) and celiac angiography. The radiological findings, differential diagnosis, and clinical significance of this anomaly are reviewed.