Infantile neuroaxonal dystrophy is a rare neurodegenerative disorder, with onset in the first or second year of life, after an interval of normal psychomotor development. It is characterized by rapid motor and cognitive regression and hypotonia evolving into spasticity. This is a retrospective and descriptive study realized over a period of 10 years [January 20-October 2023], including all patients diagnosed with Infantile neuroaxonal dystrophy in the neurology unit of the Paediatric Department of the Hassan II University Hospital of Fez. We report 7 patients with Infantile neuroaxonal dystrophy, three girls and four boys. Earliest symptoms presented between 10 and 18 months of age. The first manifestations were a psychomotor regression in all cases and pshymotor developmental delay in 5 cases. Fast rhythms on electroencephalogram (EEG) were observed in all patients. Brain imaging studies showed cerebellar atrophy, with signal hyper- intensity in the cerebellar cortex on T2-weighted images in six patients. Mutations in the PLA2G6 gene were identified in the four patients studied. Infantile neuroaxonal dystrophy is a rare and serious disease in children with a risk of generally fatal complications, but it remains a preventable disease thanks to genetic counselling.