Abstract: Autoinflammatory diseases (AIDs) are a group of genetic disorders characterized by generalized inflammatory responses and multiorgan involvement primarily caused by dysregulated innate immunity. Since the introduction of this concept, AIDs has been a rapidly advancing research field including at least 56 diseases, deepening the understanding of the interaction between innate and adaptive immunity. Despite distinct features displayed by AIDs of different categories, genetic testing remains essential for highly suspected cases. The diagnosis of undifferentiated systemic autoinflammatory diseases, omics-powered precision stratification and targeted therapy for AIDs are promising research areas in the future. This article introduces the rapid progresses in AIDs concept, mechanism, and classification. We present a summary of the characteristic clinical phenotype, as well as the current diagnostic challenges and treatment experiences, in the hope of raising the awareness of these disorders.