Another type of gender development disorder is 46, XX-DSD; most cases are SRY positive (SRY+) (90%) (McElreavey et al., 1993; Vorona et al., 2007), which usually manifests as fully differentiated male external and internal genitalia with small testes, although it occasionally manifests as SRY negative and results in unclear genitalia. The gene discussed is SRY; the disease is disorder of sexual differentiation.