There is a wide range of congenital anomalies of the spleen. Some are common, such as splenic lobulation and accessory spleen. Other less common conditions, such as wandering spleen and polysplenia, have particular clinical significance. Radiologists need to be aware of the various congenital variants of the spleen in order to recognize clinically important anomalies and to avoid mistaking less significant ones for an abnormality. In this pictorial review, the embryology of congenital anomalies of the spleen as well as their appearance on CT are described, diagnostic pitfalls are identified and complications of the anomalies are discussed.