Agenesis of the right lobe of the liver is an extremely rare congenital anomaly and usually detected by incidental cross-sectional imaging examination. Budd-Chiari syndrome is a rare cause of portal vein hypertension and hepatic failure, with the characteristic of the hepatic venous outflow tract obstruction. This article presented a clinical case of a 43 years old female diagnosed with agenesis of the right hepatic lobe combined with Budd-Chiari syndrome by ultrasonography (US) and computed tomography (CT) and conducted a literature review for imaging diagnosis of right hepatic lobe agenesis.