IgG4-related disease is a fibroinflammatory condition by infiltration of IgG4-positive plasma cells that often presents as a tumorous lesion. This disease can affect nearly every organ system. After the pancreas, the head and neck region is second most common site for presentation of IgG4- related disease such as Mikulicz’s disease, Küttner tumor. The involvement of IgG4-related disease in laryngeal lesions is extremely rare. We have experienced a case of IgG4-related disease with pseudotumor formation in the larynx that is suggestive of malignancy in radiologic findings. But the pathology findings was finally confirmed as IgG4-related disease. Oral treatment with prednisolone was initiated, and the edematous mass reduced in size without permanent functional impairment of vocal fold mobility. We report our experience with a literature review.