Leukoencephalopathies in children are a heterogeneous group of disorders affecting the white matter of the brain, and often pose a major challenge to the treating physician for the appropriate aetiological diagnosis and management. The molecular basis for the diagnosis has never been established for this disease. However, histopathologyhas been able to confirm extensive myelin loss in the aforementioned areas of the brain.We report a two-year-old Nigerian boy with clinical and radiological features of hypomyelination, and mild atrophy of the basal ganglia and cerebellum.