Various congenital anomalies may affect the spleen, starting with common anomalies, such as an accessory spleen, up to rare conditions such as a wandering spleen and polysplenia. Most of these anatomic variants have no clinical significance; they need, however, to be recognized by the radiologist as such. Awareness of these variants is important for the radiologist to interpret the findings correctly and avoid mistaking them for a clinically significant abnormality. In this review we illustrate the spectrum of congenital anomalies of the spleen and stress pitfalls and possible complications resulting from these anomalies.