SUMMARY: A 17-year-old girl presented with recurrent attacks of acute pancreatitis, associated with severe hyperglycemia and hypertriglyceridemia, despite being on intensive insulin therapy for the last 10 years. She had severe acanthosis nigricans, generalized loss of subcutaneous fat and prominent veins over extremities. The serum levels of glucose and triglyceride did not reduce significantly, even with maximally tolerated doses of metformin (2 g), pioglitazone (45 mg) and fenofibrate (160 mg), not uncommonly seen in poor rural families in West Bengal, India. A detailed dietary recall revealed a very high carbohydrate intake (70% of total calorie) with very low protein and fat intake. A switch to a very low carbohydrate (30% of total calorie) diet led to a remarkable improvement in glucose and lipid profiles (the daily insulin requirement came down by 50% and triglyceride level came down to 600 mg/dL from 950 mg/dL). A whole-exome sequencing study confirmed congenital generalized lipodystrophy type 4. A carbohydrate restriction strategy may improve difficult-to-control glycometabolic profile in lipodystrophic subjects on high-carbohydrate diet. LEARNING POINTS: Lipodystrophy should be suspected in patient presenting with hyperglycemia, hypertriglyceridemia and low BMI. A very low carbohydrate diet (30% of total daily calorie intake) may significantly improve glucose and lipid profiles in patients with lipoatrophic diabetes. Blood glucose may be the most important initial step to control hypertriglyceridemia and risk of pancreatitis in this group of patients.