The goal of treatment for CAH is to return the androgen levels to normal. This is usually accomplished through drug therapy, although surgery is an alternative. Lifelong treatment is required.

Drug therapy consists of a cortisol-like steroid medication called a glucocorticoid. Oral hydrocortisone is prescribed for children, and prednisone or dexamethasone is prescribed for older patients. For patients with salt-wasting CAH, fludrocortisone, which acts like aldosterone (the missing hormone), is also prescribed. Infants and small children may also receive salt tablets, while older patients are told to eat salty foods. Medical therapy achieves hormonal balance most of the time, but CAH patients can have periods of fluctuating hormonal control that lead to increases in the dose of steroids prescribed. Side effects of steroids include stunted growth. Steroid therapy should not be suddenly stopped, since adrenal insufficiency results.

Patients with CAH should see a pediatric endocrinologist frequently. The endocrinologist will assess height, weight, and blood pressure, and order an annual x ray of the wrist (to assess bone age), as well as assess blood hormone levels. CAH patients with the milder form of the disorder are usually effectively treated with hydrocortisone or prednisone, if they need medical treatment at all.

Females with CAH who have masculine external genitalia require surgery to reconstruct the clitoris and/or vagina. This is usually performed between the ages of one and three.

An experimental type of drug therapy—a three-drug combination, with an androgen blocking agent (flutamide), an aromatase inhibitor (testolactone), and low dose hydrocortisone—is currently being studied by physicians at the National Institutes of Health. Preliminary results are encouraging, but it will be many years before the safety and effectiveness of this therapy is fully known.

Adrenalectomy, a surgical procedure to remove the adrenal glands, is a more radical treatment for CAH. It was widely used before the advent of steroids. Today, it is recommended for CAH patients with little or no enzyme activity and can be accomplished by laparoscopy. This is a minimally invasive type of surgery done through one or more small 1 in (2.5 cm) incisions and a laparoscope, an instrument with a fiber-optic light containing a tube with openings for surgical instruments. Adrenalectomy is followed by hormone therapy, but in lower doses than CAH patients not treated surgically receive.