Atypical anti-glomerular basement membrane (GBM) disease is rare, and the atypical anti-GBM disease with membrane hyperplasia lesion is even rarer. The treatment plan for it is not clear. This article reports a case of atypical anti-GBM disease with a negative anti-GBM antibody test, but renal tissue biopsy showed “glomerular membrane hyperplasia lesions with positive IgG linearity”, which provides a reference for clinical diagnosis and treatment. The patient exhibited massive proteinuria, hematuria, edema, and renal impairment. After ruling out secondary factors, the patient was diagnosed with “atypical anti-GBM nephritis” by renal tissue biopsy, and was treated with glucocorticoids combined with cyclophosphamide, after which the patient developed a lung infection and acute left heart failure, and received regular hemodialysis treatment. Then the patient progressed to the stage of end-stage renal disease and continued to receive regular hemodialysis treatment.