Background: Suid herpesvirus type 1 (SHV1) is a type of neurotropic virus able to infect various species. However, the clinical cases of human SHV1 encephalitis are still rarely reported, and the clinical characteristics, treatment, and prognosis of human SHV1 encephalitis are still unclear.Methods: In this study, we reported 2 cases of human encephalitis associate with SHV1 infection, and reviewed the other 18 cases from the literatures. A total of 20 cases with human SHV1 encephalitis were summarized and re-analyzed.Results: Nineteen of 20 patients had a history of swine-related occupational exposure before illness onset. All patients initially presented with influenza-like symptoms, and then quickly developed seizures, disturbed consciousness, and respiratory failure. The results of cerebrospinal fluid (CSF) indicated aseptic or viral infection. MRI findings of SHV1 encephalitis were prone to distribute in temporal-frontal and insular cortex, which was similar to the pattern of herpes simplex virus encephalitis. Some cases with disease progression and poor prognosis might expand to thalamus, basal ganglia and brain stem. Metagenomic next-generation sequencing (mNGS) revealed that all patients had unique SHV1 sequences with variable reads in the CSF.Conclusions: SHV1 is a zoonotic pathogen that can cause a new type of human viral encephalitis, characterized by acute, fulminating and catastrophic central nervous system infection. Currently, there are no available approved treatments for the encephalitis, but it is possible to diagnose encephalitis quickly by mNGS.