Ambiguous genitalia True hermaphroditism 2009-09-15T08:28:15Z HP:0003242 The presence of both ovarian and testicular tissues either in the same or in opposite gonads. Affected persons have ambiguous genitalia and may have 46,XX or 46,XY karyotypes or 46,XX/XY mosaicism. UMLS:C0266361 In true hermaphroditism, the testicular tissue must have well developed seminiferous tubules, and the ovarian tissue primordial follicles. The external genitalia are usually associated with variable degrees of sexual ambiguity, depending on the amount of functioning testicular tissue. Internally, a uterus is almost always present, but the development of Mullerian and Wolffian structures is variable and generally depends on the adjacent gonad. Usually there is a vas deferens on the side of the testis, and a Fallopian tube on the side of the ovary. Both types of ducts can occur next to an ovotestis. An ovotestis is the most common gonad found in true hermaphroditism and can be coded with HP:0012861. HP:0010459 Testicular and ovarian tissue present SNOMEDCT_US:18978002