BACKGROUND፡ Vanishing white matter disease is one of the most prevalent inherited childhood leukoencephalopathies. The disease is characterized by chronic, progressive and episodic deterioration with ataxia and spasticity.CASE PRESENTATION: Here, we report a 15-month-old female child from Dire-Dawa, eastern part of Ethiopia, who presented with regression of developmental milestones and truncal ataxia since her age of 11 months following a febrile illness that occurred one month earlier. Magnetic resonance imaging of brain is suggestive of vanishing white matter disease. CONCLUSIONS: We believe this case report will increase curiosity, awareness and knowledge of health professionals in Ethiopia and sub-Saharan Africa working with children in early consideration and the diagnosis of the disease.KEYWORDS: Vanishing White Matter Disease, Child, Ethiopia