Wolfram syndrome is a rare neurodegenerative genetic disease, its prevalence is 1/700 000 cases. There are three known types. The first type is due to the mutation of WFS1 (4p16.1) gene. This results in the appearance in the first decade of life of diabetes mellitus type 1, diabetes insipidus, sensorineural hearing loss, bilateral optic atrophy and neurological damage signs. The second type is due to the mutation of CISD2 (4q24) gene. It differs from the first in the absence of diabetes insipidus. Types 1 and 2 are autosomal recessive. The third type or Wolfram-like syndrome is autosomal dominant, differs from the first two by the late onset of optic atrophy and diabetes mellitus type 1 (after adolescence) and hearing impairment is not always present. We report the first documented case of Wolfram syndrome at the University Hospital of Brazzaville in a nine year old girl.