Splenic-gonadal fusion is a rare congenital anomaly, in which splenic tissue is connected to the gonad. It is almost always on the left side, is difficult to be recognized, and unfortunately the final diagnosis is confirmed after an unnecessary orchiectomy in one-third of the cases. We report a case of splenic-gonadal fusion in a 6-year-old boy, referred for a palpable mass at the left testicle. Ultrasound and MRI were performed; and the final diagnosis was confirmed by Tc-99m colloid scanning. The child was operated, orchiectomy was avoided, the mass was removed, and pathology confirmed normal splenic tissue.