Background: Simultaneous occurrence of steroid-sensitive nephrotic syndrome (NS) and Type 1 diabetes mellitus (DM) in children is rare. Clinical description: A 21-month-old boy presented with generalized swelling with hypertension, with no significant illness in the past. Mother had type 1 DM from 11 years of age. Management and Outcome: Investigations revealed nephrotic range proteinuria, hypoalbuminemia along with raised blood sugar levels without ketoacidosis. Raised HbA1C and positive anti-glutamic acid decarboxylase confirmed type 1 DM. Ultrasonography detected a unilateral renal hypo-dysplastic kidney, which was non-functional on nuclear scan; vesico-ureteral reflux being ruled out by micturating cysto-urethrography. A therapeutic trial of steroids resulted in remission of proteinuria, insulin being titrated for glycemic control. Genetic analysis was negative and child remained relapse-free till last follow-up at one year. Conclusion: Nephrotic syndrome and DM may present concurrently. In the absence of genetic abnormality, and the presence of response to steroids, diabetic nephropathy as well as secondary focal segmental glomerulosclerosis are possibly ruled out.