RESUMEN
Temporal bone encephalomeningoceles are uncommon clinical entities that can be challenging to diagnose and treat. Common presenting complaints include symptoms of aural fullness, hearing loss, clear otorrhea and meningitis. Common etiologies are chronic middle ear disease and trauma. A significant proportion of cases are spontaneous, which occurs in relation to benign intracranial hypertension. Normal pressure hydrocephalus associated with temporal bone encepahomeningoceles has not been previously described. The authors present an unusual case of spontaneous right sided temporal bone encephalomeningocele associated with symptomatic normal pressure hydrocephalus. Surgical repair of the defect was successful. The diagnostic and management approach to this unique case are discussed.