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Ovotesticular disorder denotes the presence of both ovarian and testicular tissue in the same patient. There may be an ovary on one side and a testis on the other or, more commonly, ovotestes situated bilaterally or unilaterally. The differentiation of the genital tract, the appearance of the external genitalia, and the development of secondary sexual characteristics are all variable. This depends on relative gene expression and hormone secretion, and histology of the gonads can include a mixture of ovarian and testicular tissue. The external genitalia are often ambiguous, but a uterus is almost invariably present, and 60% of patients have a 46,XX karyotype. Pregnancy is possible [41].