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Background: 11beta-hydroxylase deficiency (11βOHD) is clinically presented with external genitalia virilization in girls and precocious puberty in boys. Low renin hypertension occurs in both sexes. Early diagnosis and treatment of hypertension can prevent complications.
Objectives: To provide a practical management for controlling hypertension in patients with 11-beta-hydroxylase deficiency.
Methods: We described a 4.5 years old girl of 46.XX, who presented with ambiguous genitalia at birth and hypertension later in follow-up. The patient had received the appropriate dosage of hydrocortisone and the level of 17-hydroxy progesterone was within the acceptable range but the hypokalemia persisted. Both hypertension and hypokalemia were normalized when spironolactone was added.
Results: Blocking mineralocorticoid receptor treat hypertension in 11-beta-hydroxylase deficiency.
Conclusions: Intermittent measurement of blood pressure is necessary for patients with 11βOHD. In these patients, spironolactone is effective in treating mineralocorticoid-mediated hypertension and hypokalemia by blocking mineralocorticoid receptors.

Keywords: Congenital adrenal hyperplasia, 11 beta-Hydroxylase, Hypokalemia, Antihypertensive agents

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