Mauriac syndrome is a rare complication of type1 Diabetes Mellitus (DM) with poor glycemic control. This is a glycogenic hepatopathy with cardinal features of growth failure or delayed growth and puberty, cushingoid appearance with moon facies and hepatomegaly. These clinical features are mainly due to poor insulin compliance or sub optimal diabetic management. Now a days its incidence is decreasing due to newer insulin formation and blood glucose control. The mainstay of treatment is strict glycemic control with insulin therapy. Early recognition and management of this syndrome may improve the outcome of this syndrome.We present an unusual presentation of 7yr old male child with 4 years history of type 1 diabetes mellitus with poor insulin compliance and was diagnosed to have Mauriac syndrome and started on regular insulin therapy.