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Splenogonadal fusion is a rare congenital abnormality that affects mostly the male gender, and may present as cryptorchidism. A pre-operative diagnosis is rare and difficult, and may be mistakenly diagnosed as a gonadal tumor of malignant nature leading to unnecessary orchiectomy. The authors present two case reports concerning the two types of splenogonadal fusion described, with a subsequent literature review, alerting for the clinical presentations and available complementary exams.