Metabotropic Glutamate Receptor 1 (mGluR1) encephalitis is a rare encephalitis characterized by ataxia, neuropsychiatric symptoms, dysarthria and cognitive impairment. This disease process has been described in several adult patients and has been associated with paraneoplastic syndrome in Hodgkin's lymphoma and other cancers as well as parainfectious and underlying autoimmune etiologies. However, only two cases of anti-mGluR1 encephalitis in children have been reported in the literature. The underlying etiology of one case was not provided but post-infectious disease has been reported. Here, we report the first case of anti-mGluR1 encephalitis in a child with a presumed "idiopathic" basis.