Congenital generalized lipodystrophy is a rare genetic disorder characterized by the near-total absence of body fat. Individuals with this condition typically have a normal or increased muscle mass and may experience metabolic issues, such as insulin resistance and high triglyceride levels. This can lead to complications like diabetes and fatty liver disease. The condition is caused by mutations in specific genes, including AGPAT2 and BSCL2. Diagnosis is usually made through clinical evaluation and genetic testing. Treatment focuses on managing symptoms and complications, often involving lifestyle changes, medications, and regular monitoring of metabolic health.