Abstract: Anti-glomerular basement membrane disease is an autoimmune disease with low incidence but severe clinical symptoms. With the development of diagnosis and related technologies, it is found that in patients with anti-glomerular basement membrane disease, the clinical manifestations and laboratory examination results of some patients are different from those of typical patients in the process of clinical diagnosis. The pathogenesis of such patients with anti-glomerular basement membrane disease are not clear and the clinical manifestations are not typical, it brings some difficulties to clinical diagnosis and there is no uniform standard for its treatment at present. This article will review the research progress of atypical anti-glomerular basement membrane disease.