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This is a case report of a young male with poorly controlled type 1 diabetes mellitus who presented with the clinical features of diabetic ketoacidosis. On examination, he had severe growth retardation, elevated liver enzymes, advanced diabetic eye disease, and other diabetes-related microvascular complications. Along with the other clinical features, the patient was diagnosed as a case of Mauriac syndrome. Mauriac syndrome, initially described by Mauriac in 1930, is one of the causes of growth retardation and elevated liver enzymes in poorly controlled diabetic patients. Patients with poorly controlled type 1 diabetes must be closely observed for sexual maturation and growth. An early intervention with optimal therapy to bring glycemic control combined with a close follow-up would help avoid such late complications.