From 1960 to 1990, some Chinese dermatologists noticed that a small number of patients presenting with granulomatous inflammation on the face eventually died of encephalitis. In 2001, Dr. Tianwen Gao summarized clinical and pathological characteristics of these patients and proposed the disease name "fatal bacterial granuloma after trauma", and Propionibacterium acnes was assumed to be the most probable pathogen at that time. In 2018, the disease was confirmed to be Balamuthia mandrillaris infection involving the skin and brain tissues. This review summarized the history of research on Balamuthia mandrillaris infection in China, as well as its clinical, pathological characteristics and treatment methods.