Epidermoid cysts, also known as epidermal and keratin cysts, or congenital cholesteatomas are benign congenital non-neoplastic tumors, rarely observed in the brain of humans and some animal species (dogs, horses, mice, and rats). Histologically, they are composed of laminated, concentrically arranged keratin surrounded by a thin layer of stratified squamous epithelium. We describe a case of intracranial epidermoid cyst in a 6-year-old mixed-breed male cat in southern Brazil. The patient presented central vestibular syndrome. Given the poor prognosis and the fact that it belonged to a shelter with many dogs and cats, the owner requested euthanasia, and a thorough post-mortem examination was conducted immediately after death. The definitive diagnosis was based on histopathological findings. To the best of our knowledge, this is the first report of an intracranial epidermoid cyst in a cat.