Acquired generalized lipodystrophy is a rare disease, and often follows autoimmune disease, prodromal infection, HIV infection. The clinical characteristics are generalized absence of fat, insulin resistance, diabetes mellitus, absence of ketosis, elevated basal metabolic rate, severe hypertriglyceridemia, and hepatomegaly. Here we experience and report a case of 16-year-old female patient who has clinical features of acquired generalized lipodystrophy with severe insulin resistant diabetes mellitus without any prodromal states.