Objective To explore the clinical characteristics and treatment strategies of infective endocarditis caused by Bartonella vinsonii subsp. berkhoffii (Bvb). Methods Clinical diagnosis and treatment of a 25-year-old male patient with infective endocarditis caused by Bvb in China was reported, combined with literatures about three similar cases reported abroad were summarized and analyzed. Results All 4 patients were young and middle-aged males with a history of close contact with canines in the past one year. The main symptoms were chest pain, fatigue, and dyspnea, accompanied by cerebrovascular accidents and severe anemia, as well as the formation of heart valve vegetations and valve function impairment. Multiple blood cultures were negative, 2 and 3 cases were confirmed to be infected with Bvb through metagenomic next-generation sequencing (mNGS) of pathogenic microorga-nisms and polymerase chain reaction respectively. All patients underwent surgical treatment due to heart failure, and all survived after surgery and targeted anti-infective treatment. Conclusion This case report is the first case of Bvb infective endocarditis in China. Patient’s diagnosis is confirmed by serum indirect immunofluorescence assay and mNGS, combination of surgery and anti-infective treatment has achieved ideal effect.