Nasal septum IgG4-related disease is a chronic inflammatory and fibrotic disease. The main manifestation is nasal septal mass with or without involvement of other organs. At present, the pathogenesis of the disease is unknown, and it has a certain degree of bone invasiveness, so it is easy to be misdiagnosed as Malignant tumors. This article reports the diagnosis and treatment of a case of IgG4 related diseases of the nasal septum. After surgical excision of paraffin pathology, the diagnosis was confirmed, and then maintenance treatment with hormones combined with immune preparations. After the follow-up for nearly 2 years, there was no recurrence of the nasal septum mass, and no other organs were involved. Clinically, for space-occupying lesions of unknown nature in the nasal cavity, peripheral blood IgG4 should be included in the routine primary screening method, pathology should be added to immunohistochemistry to exclude other lesions, and early diagnosis and treatment should be achieved as soon as possible.