In select cases of type 1 diabetes mellitus (DM1) the pancreas transplantation has been shown to ameliorate the disease, to reduce the need for exogenous insulin and normalize glycosylated hemoglobin (A1c) levels. The efficacy of this therapy in Mauriac Syndrome (SM) is not yet well established. We report a patient with MS treated with intensive insulin therapy, physical activity program, nutritional and psychological assistance, with persistently elevated fast glycemia and A1c levels, inadequate lipid profile and decreased IGF-1 (insulin like growth factor) levels. Due to a poorly metabolic control, pancreas transplantation was indicated. After one year follow up, the patient had no symptoms and showed persistent insulin independence with fast glucose <110 mg/dl, normal lipid profile and IGF-1 levels and significant decrease in A1c (4.6%). The pancreas transplantation improved diabetes control and promoted better quality of life for this patient. Pancreas transplantation proved to be an effective treatment strategy in patients with MS, improving their clinical and biochemical derangements. In this report we present the first case of MS controlled by pancreas transplantation registered in the indexed medical literature, as an alternative therapy in this group of patients.