Karyotype 46 XX Physical appearance - male pattern Precocious pubey at 2-4 yrs. of age. Uterus, cervix and fallopian tubes are present (bcz 44 XX) - Mullerian duct development is normal. Metabolic problems: Hyponatremia - aldosterone deficiency Hyperkalemia Hypotension Diagnosis: 1) USG Normal 2) Barr body present 3) Biochemistry - 17 hydroxyprogesterone || > 800 ngm/dl. Management: DOC - Dexamethasone - to pregnant women and fetus both having CAH. Hydrocoisone - pregnant women alone having CAH but fetus is normal. Neonate with CAH. Labioscrotal folds may be separated to folds may be fused at the midline, giving an appearance of a scrotum Labioscrotal folds with increased pigmentation suggest the possibility of increased coicotropin levels as pa of adrenogenital syndrome