We describe a woman with acromegaly who had acanthosis nigricans and hirsutism. Serum growth hormone (GH) and testosterone levels were markedly elevated. Standard oral glucose tolerance test (OGTT) showed a diabetic curve and no suppressed GH levels. Fasting insulin levels were very high while plasma glucose levels were not hypoglycemic. Insulin tolerance test revealed blunted hypoglycemic response. Acanthosis nigricans was present in the right axilla and face. Ultrasonogram demonstrated bilateral polycystic changes in the ovaries. From the above findings this patient's condition is characteristic of a very rare syndrome consisting of acromegaly, polycystic ovaries (PCO), hyperandrogenism, hirsutism, insulin resistance and acanthosis nigricans.