OBJECTIVE To report a case of Mauriac syndrome in a young child with poorly controlled type 1 diabetes mellitus. METHODS We describe the typical features of Mauriac syndrome and review the clinical, laboratory, and ultrasound findings and follow-up results in our current patient. RESULTS Hepatomegaly, growth impairment, and cushingoid features characterize Mauriac syndrome. Most frequently, it is seen in adolescents and young adults with a history of poor glycemic control. In our current patient, a 3-year-old boy with type 1 diabetes mellitus of 2 years' duration, Mauriac syndrome developed in the context of a disrupted social environment. His liver enzyme derangement, hepatomegaly, and growth improved once euglycemia was achieved. CONCLUSIONS Although Mauriac syndrome is currently uncommon, close monitoring of children with type 1 diabetes is important to maintain optimal glycemic control and prevent such an occurrence.