Hypomorphic mutations in RAG1 or RAG2 with partial activity of RAG are associated with (a) Omenn syndrome (OS) with oligoclonal, activated, and anergic autologous T cells that infiltrate target tissues; hypereosinophilia; and high IgE levels (12); (b) atypical or leaky SCID (AS) with the presence of residual autologous T cells and without clinical manifestations of OS (6); and (c) delayed-onset combined immunodeficiency with granulomas or autoimmunity manifestations (CID-G/AI) (13). Here, RAG1 is linked to Autoimmunity.