Abstract: Type Ⅰ interferon (INF-Ⅰ) is a group of cytokines that mediate antiviral and antineoplastic immunity, and its role in the pathogenesis of autoinflammation and autoimmune diseases has attracted more and more attention in recent years. Increased INFⅠ is observed in the context of viral infections, autoimmunity, and interferon therapy, resulting in renal injury through direct and indirect pathways. Among them, there are also a considerable number of patients, in which the elevated levels of INF-Ⅰ are associated with abnormal function of genes regulating INF-Ⅰ signaling pathway, also known as type Ⅰ interferonopathies. Studies have shown that these renal-involved patients share distinct histopathological features, such as collapsing glomerulopathy, inflammatory-proliferative pattern, vascular lesions or tubuloreticular inclusions. The development of genomic medicine has provided us with new insight into precise diagnosis and treatment for these patients.