Not XX, XY, Klinefelter, or Turner one in 1,500–2,000 births (0.07–0.05%)[138]
Klinefelter syndrome (47,XXY) one in 1,000 births (0.10%)
Turner syndrome (45,X) one in 2,710 births (0.04%)[148]
Androgen insensitivity syndrome (46,XY) one in 13,000 births (0.008%)
Partial androgen insensitivity syndrome (46,XY) one in 130,000 births (0.0008%)
Classical congenital adrenal hyperplasia (46,XY or 46,XX) one in 13,000 births (0.008%)
Late onset adrenal hyperplasia (46,XY or 46,XX) one in 10,000–20,000 births (0.01–0.005%)[149]
Vaginal atresia (46,XX) one in 6,000 births (0.017%)
Ovotestes (45,X/46,XY mosaicism) one in 83,000 births (0.0012%)
Idiopathic (no discernable medical cause; 46,XY or 46,XX) one in 110,000 births (0.0009%)
Iatrogenic (caused by medical treatment, e.g., progestin administered to pregnant mother; 46,XY or 46,XX) No estimate
5-alpha-reductase deficiency (46,XY) No estimate
Mixed gonadal dysgenesis (45,X/46,XY mosaicism) No estimate
Müllerian agenesis (of vagina, i.e., MRKH Syndrome; 46,XX) 1 in 4,500–5,000 births (0.022–0.020%)
Complete gonadal dysgenesis (46,XY or 46,XX or 45,X/46,XY mosaicism) one in 150,000 births (0.00067%)