IgG4-related disease is a newly recognized systemic fibro inflammatory disorder that affects the sino-nasal region.It is a rare and emerging entity that can present with bony and soft-tissue invasion,the final diagnosis of this disease mainly depends on pathological examination and majority of patients receiving corticosteroids responded very well to treatment.Thus,Our goal was to highlight the sino-nasal presentation of this unique disease and to review previously reported cases from 2010 to 2016.We hope that clinical physicians to enhance understanding of the disease in order to ensure early diagnosis and early intervention to prevent serious injury and fibrosis of organs.