Splenogonadal fusion is a rare congenital malformation that involves an abnormal connection between the spleen and the gonad or mesonephric derivatives. It manifests itself as a mass consisting of splenic and testicular or ovarian tissue. Splenogonadal fusion has been classified into two types; continuous, where there is a direct connection between spleen and gonad; and discontinuous, where ectopic splenic tissue is attached to the gonad, but there is no connection to the spleen. Many cases had an associated other anomalies either genital or systemic. Knowledge about the existence of such an uncommon entity is essential even to be suspected preoperatively as the appearance at exploration can be misleading as a malignant mass and often results in unnecessary gonadal removal.
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