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. 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. 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. 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.