Enhanced type Ⅰ interferon (IFN-Ⅰ) signaling pathway is observed in the setting of viral infections, autoimmune abnormality, type 1 interferonopathies, etc. All these IFN-Ⅰ-related disorders can cause renal dysfunction and share common histopathological features. The underlying mechanism lies in that IFN-Ⅰ can mediate kidney injury either by directly damaging several renal cell types (including podocytes, mesangial, endothelial and parietal epithelial cells) or promoting immune-mediated glomerular injury. This emerging spectrum of kidney diseases is defined as IFN-Ⅰ-related kidney disorders. This paper reviews the pathogenesis, etiological classification, histopathological characteristics, and diagnosis and treatment of the diseases.