Wolfram syndrome is a rare diffuse neurodegenerative disorder also known as DIDMOAD due to its characteristics of diabetes insipidus, diabetes mellitus, optic nerve atrophy and deafness. It is also associated with a wide variety of abnormalities of the central nervous system, urinary tract and endocrine glands. It may be familial or sporadic. Imaging findings include absence of the short T1 nature of the pituitary posterior lobe, atrophy of the optic nerve, chiasma, and tracts. Atrophy of the brain stem and cerebellum has also been reported. We describe a 15-year-old boy and an 11-year-old girl with Wolfram syndrome who were siblings from a diabetes mellitus family. They received regular insulin control at our hospital and had symptoms of frequent urinary tract infection and diabetes insipidus. Magnetic resonance imaging revealed marked pons and cerebellar atrophy. Optic nerve and chiasma atrophy was also noted.