The authors report the case of a 23-year-old woman with a virilizing lipoid cell tumor of the ovary. The patient developed precocious puberty at age 4 and from age 5 to 10 years was treated weekly with intramuscular medroxyprogesterone acetate. She began to menstruate spontaneously at age 13, but developed hirsutism, acne, and irregular menses at age 15 followed by secondary amenorrhea at age 20. Steroid analyses demonstrated elevated peripheral plasma levels of testosterone, dihydrotestosterone, androstenedione, 17 alpha-hydroxyprogesterone, progesterone, desoxycorticosterone, estrone, and estradiol. Catheterization of the adrenal and ovarian veins revealed increased secretion of testosterone, dehydroepiandrosterone, androstenedione, 17 alpha-hydroxyprogesterone, progesterone, desoxycortcosterone, and corticosterone, 11-desoxycortisol, cortisol, estrone, and estradiol from the right ovarian vein. At surgery a right ovarian lipoid cell tumor was removed. Incubation studies with the tumor tissue confirmed the presence of gonadal and adrenocortical steroids. The tissue also contained high-affinity human chorionic gonadotropin-luteinizing hormone binding sites with normal binding affinity.