Introduction ▼ Splenogonadal fusion is a rare congenital anomaly and is the result of an abnormal association of splenic and gonadal tissue during gestation. The etiology remains unclear. Splenogonadal fusion may originate in the early embryonal period, during which the stomach rotation causes proximity between the different anlages. It is almost always found on the left side, given the anatomical proximity of the spleen and the left gonad during embryogenesis [R. Lopes et al. Einstein (Sao Paulo) 2012; 10: 92-95, C. Lin et al. J Urol 2010; 184: 332-333]. The condition is an important differential diagnosis to other scrotal masses, particularly testicular cancer, which in unclear cases leads to unnecessary orchiectomies with benign pathologic results.