Splenogonadal fusion (SGF) is a rare congenital anomaly in boys and girls. It commonly presents as a testicular mass treated with an unnecessary orchiectomy. We present 3 cases of SGF diagnosed in adulthood and discuss the published literature focusing on several aspects of this rarity. These cases of SGF of both types presented with the usual complaints. In our first case, orchiectomy was performed since the left testis was hypoplastic and testicular neoplasm could not be ruled out. The testicles could be preserved in the latter 2 cases. The second case is the continuous-type SGF associated with contralateral testicular aplasia and, to our knowledge, this is the first reported case of continuous SGF associated with testicular aplasia. Knowledge of this entity may help prevent unnecessary orchiectomy.

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