Introduction
The CYP17A1 gene encodes for the P450c17 enzyme which catalyzes two key enzymes: 17-alpha hydroxylase and 17,20-lyase. These enzymes are expressed mainly in the gonads and in the adrenal glands, and play a pivotal role in the biosynthesis of aldosterone, cortisol and sex steroids. The genetic defects of this gene result in a rare form of congenital adrenal hyperplasia (CAH).The main clinical determinants of this disorder are: hypertension, hypokalemia and disorders of sex development (DSD)[1]. Herein we report the intriguing case of a 42-year-old woman presenting with grade three hypertension, severe hypokalemia and primary amenorrhea, which revealed to be the complete form of 17 alpha-hydroxylase deficiency (17 OHD). We also discuss the challenging therapeutic approach as well as the outcomes and the follow-up of this patient.