Agenesis or hypoplasia of the liver lobes are very uncommon developmental anomalies and can be recognized on CT examinations. These are usually asymptomatic and incidental finding, but they cause an altered topography and anatomical relationship of the upper abdominal organs. This can lead to various complications, particularly during the surgical or interventional procedures involving the liver or gallbladder. The CT appearance of these anomalies and their clinical implications are presented in this article, and differential diagnosis from acquired liver atrophy due to various pathological processes is also discussed.