Leukodystrophies are rare degenerative disorders that involve mainly the white matter of the brain. It is associated specific clinical features including neurological deterioration following a period of normal development, predominant involvement of the motor function at least initially, and absence of convulsions or myoclonus. Leukodystrophy with oligodontia is characterized by progressive ataxia beginning during infancy, a pyramidal syndrome (spasticity, hyperreflexia), and dental agenesis.