Background: Wolfram syndrome is a rare neurodegenerative autosomal recessive disorder. That occurs in association with diabetes mellitus, diabetes insipidus, and optic nerve atrophy, hearing loss, urinary tract infection and nervous complication.

Case: The present report belong to a 24 years old girl who was effected by diabetes mellitus in 10 years old & has been effected by Diabetes insipidus, recurrent UTI, hearing loss and other type of disorders in 20 years old.

Conclusion: Strict control of blood glucose and regular fundoscopy, for early detection of optic atrophy are important. Step in management of this syndrome and quality of life is improved with hearing and vision aid tools.