Endocrine studies were made on 4 women with ovarian Sertoli-Leydig cell tumors of varying degrees of differentiation. Clinically, all 4 patients showed evidence of increased androgen production, manifested by either hirsutism or virilization. The calculated ratios of steroid hormones between the affected ovarian venous values and peripheral values for testosterone (T), androstenedione (A), and dehydroepiandrosterone (DHEA) in 3 patients, were 1.4-18.6, 4.2-24.4, and 3.7-10.0, respectively. The peripheral levels of the hormones before salpingo-oophorectomy in all the patients were T: 0.97-45.0 ng/ml; A: 2.92-114.0 ng/ml; and DHEA: 13.0-20.9 ng/ml. In the 3 juvenile patients, elevated basal levels of LH and normal or subnormal levels of FSH (high LH:FSH ratio) were found. In a 12-year-old patient with 1,900 g tumor composed predominantly of Sertoli cells with lipid storage, the peripheral serum levels of T and estradiol (E2) were 114 ng/ml and 1890 pg/ml, respectively. The present data suggest that: 1) androblastomas have a biosynthetic capacity for androgen, estrogen, or both in which both the delta 5 and delta 4 pathways are involved, and 2) little correlation exists between the endocrine function and cellular composition of the tumors or their degree of differentiation.