Splenic-gonadal fusion is an extremely rare congenital anomaly that results from fusion between the splenic and gonadal anlage during embryonic development. Approximately 90 cases have been reported in the literature. The condition has been divided into two major subgroups: continuous splenic-gonadal fusion in which a continuous cord-like structure connects the spleen and the gonadal-mesonephric structures, and discontinuous splenic-gonadal fusion in which no such connection exists. We report a case of discontinuous splenic-gonadal fusion in an adult women with no evidence of other anomalies or malformations, the fifth reported case of splenic-gonadal fusion occurring in a female. To our knowledge, this is the first case of discontinuous splenic-gonadal fusion reported in a female patient, and the second case of splenic-gonadal fusion reported in an adult woman.