Since the mechanisms of monogenic autoinflammatory diseases have been investigated,it has been realized that new and surprising pathways can cause this group of diseases. Type-I interfer- ons (IFNa/b) are a family of cytokines responsible for the expression of hundreds of genes in the immune response. Therefore, the initiation, transmission and termination of the IFNa/b-mediated response is reg- ulated by strict control mechanisms. Mutations in the processing, recognition, or feedback mechanisms of the IFNa/b pathway may result in excessive IFNa/b response. As a result, clinical findings specific to the disease may be seen depending on the location of the mutation and the type of inheritance as well as some common findings. In this review, pathogenesis, clinical findings and treatment of diseases called Type-I Interferonopathies (Aicardi-Goutières Syndrome, STING Associated Vasculopathy of Infacy- SAVI, Spondyloenchondromatosis-SPENCD, Singleton-Merten Syndrome and other interferonopathies) will be described. Proteasome pathway disorders with similar outcomes have not been discussed in this review.