A rare case of adrenocortical adenoma in a 7-month-old female infant with congenital hemihypertrophy of left leg is described. Only 7 similar cases including the present one, are on record. The patients ranged in age from 7 weeks to 45 years. The male:female ratio was 3:4. The adrenal tumors were often detected after CH had become evident. Large functional tumors often produced symptoms of hirsutism, virilization, and precocious puberty. CT and ultrasound are necessary for early detection and prompt treatment.