We report a case of splenogonadal fusion masquerading as a testicular tumor and review the etiology, the pathogenesis and the management of this rare disease. A 20-year-old male patient presented with clinical and radiological findings of a left testicular mass for which he underwent a left radical inguinal orchidectomy. Histopathology of the removed testicle revealed no tumor. The gonad was fused to ectopic splenic tissue, the later forming a distinct encapsulated mass attached to the lower pole of the testicle inside the tunica vaginalis. Splenogonadal fusion, although a rare condition, may account for a non-malignant testicular mass and should be suspected in young patients with other congenital anomalies.