Abstract: Stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) is a rare auto-inflammatory disease caused by gain-of-function mutations in human STING1 gene. With the increasing number of SAVI patients reported in recent years and the establishment of related mouse disease models, researchers have gradually deepened their understanding of the pathogenesis and treatment strategies of SAVI. This paper systematically reviews the reported cases of SAVI to briefly describe their pathological mechanisms and treatment strategies, with a view to providing some reference for the clinical treatment of this rare disease.