Granulomatous Meningoencephalomyelitis (GME) is a presumed autoimmune, non-infectious inflammatory disease that can affect the brain, spinal cord, and meninges. It is considered a meningoencephalomyelitis of unknown origin (MUO) along with other diseases such as Necrotizing Leukoencephalitis (NLE) and Necrotizing Meningoencephalitis (NME). Patients with GME present with non-specific clinical signs associated with the part of the central nervous system that is implicated by the disease. A definitive diagnosis is based on histologic evaluation of neural tissue; hence treatment is often implemented based on a presumptive diagnosis using signalment, history, clinical signs, magnetic resonance imaging, and cerebrospinal fluid analysis. Treatment includes immunomodulatory and analgesic medications. The aim of this paper is to provide an overview of the differential diagnoses, clinical signs, diagnostics, treatment options, and prognoses for canines diagnosed with GME.