Anti-IFN-γ autoantibodies cause adult immune deficiency, leading to opportunistic bacterial infection, which is a recent medical issue. Nontuberculous mycobacterial (NTM) infection is the most common infection in patients with positive anti-IFN-γ autoantibodies, and NTM infection relevant to anti-IFN-γ autoantibodies has been increasing in recent years. This article reviews the pathogenic mechanism, NTM species, clinical manifestations and immunoregulation adjuvant therapy of anti-IFN-γ autoantibody-associated NTM infection, aiming to help clinicians better understand this disease, avoid misdiagnosis and missed diagnosis, as well as conduct rational treatment. Methods Results Conclusion