Wolfram syndrome (WFS) is a rare autosomal recessive disorder which manifests with diabetes mellitus, diabetes insipidus (DI), optic atrophy, and deafness. Central DI is a late presentation of the disease which may rarely present with renal tract anomalies and recurrent urinary infections instead of classical symptoms. We report a family of three siblings with WFS with early onset of central DI and renal tract abnormalities indicating need to suspect and manage these conditions early.