Background: Pseudorabies virus (PRV) is a common pathogen found in pigs. The pathogenicity of PRV in humans is under researched and there are few confirmed cases of PRV infections in humans, which has led to a lack of clinical consensus.Methods: We presented a case of viral encephalitis caused by PRV in China. We performed a systematic review of the literature to investigate the clinical features and prognosis of PRV encephalitis and included 12 patients with PRV encephalitis.Results: All the patients had a history of direct or indirect contact with living pigs or pork before the onset of the disease, accompanied by prodromal symptoms, such as fever and headache. They presented with a series of lesions involving the central nervous system (CNS) and respiratory system, such as acute encephalitis syndrome, respiratory failure, retinitis, or endophthalmitis.Conclusions: The differential diagnosis of an acute attack of CNS infection should include PRV encephalitis, which should be diagnosed by a head magnetic resonance imaging (MRI), fundus examination, and cerebrospinal fluid next-generation sequencing. Intravenous immunoglobulin, glucocorticoid, antiviral, and symptomatic support treatment should be administered as early as possible to improve the prognosis.