Rabson mendenhall syndrome is a rare autosomally recessive inherited insulin resistant disorder. This is characterized by growth retardation, dysmorphisms, lack of subcutaneous fat, acanthosis nigricans, enlarged genitalia hypertrichosis, premature and dysplastic dentition, coarse facial features, paradoxical fasting hypoglycemia and postprandial hyperglycemia, extreme hyperinsulinemia, protracted course, and eventual development of ketoacidosis. We report a male patient with all the features of Rabson-Mendenhall syndrome from our institute.