With some remaining P450c17 enzymic activity, clinical presentations of 46,XX patients with p17OHD include spontaneous menarche, irregular menses, oligomenorrhea or primary amenorrhea, spontaneous breast development, sparse or absent axillary and pubic hairs, juvenile genitalia, and the presence/absence of mild hypertension or hypokalemia. The gene discussed is CYP17A1; the disease is Hypokalemia.