Granulomatous Meningoencephalitis is presumed to be an autoimmune, non-infectious inflammatory disease that affects the brain, meninges and/or spinal cord. Patients present with non-specific signs related to the region of the central nervous system that is affected. Definitive diagnosis is rarely made antemortem due to the need for a lesion biopsy. Nevertheless, treatment with immunomodulatory drugs is implemented based on a presumptive diagnosis using MRI findings, clinical signs, signalment and CSF analysis. The aim of this paper is to provide a concise overview to the clinical signs, diagnosis and treatment of Granulomatous Meningoencephalitis as well as provide a brief review of the skin condition Ichthyosis, a coincidental finding in our patient.