We report a 14-year-old boy who presented with meningoencephalitis. Other features particularly auditory, vestibular, and ocular lead to the diagnosis of Cogan's syndrome. Treatment with prednisolone resulted in a rapid improvement and recovery of his hearing. Cogan's syndrome is a rare primary vasculitis, characterized by ocular, auditory, and vestibular symptoms, which can have significant morbidity and mortality. Presentation with a meningoencephalitic picture is unusual. Increased awareness of its clinical features among pediatricians and pediatric neurologists should lead to earlier diagnosis and increased recognition of the serious systemic manifestations. Early use of prednisolone can prevent hearing loss and can also be useful in treating the other vasculitic manifestations.