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IgG4-related diseases are immune-mediated diseases associated with fibroinflammatory lesions and are known to invade almost all organs of the human body. In the head and neck area, it rarely occurs in the nasal cavity and sinuses. Diagnosis is made by combining clinical features, serological, imaging, and histopathological findings, and glucocorticoids are known as the main treatment. In general, surgical resection is not regarded as a curative treatment option. Here we report a case with literature review of IgG4-related diseases with sino-nasal involvement experiencing prominent nasal symptom relief and remission with surgical treatment, which did not respond well to oral steroid in a 70-year-old male patient.