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Objective Immunoglobulin G4 (IgG4) related sclerosing disease (RSD) of the paranasal sinuses is a rare lesion of dense lymphoplasmacytic tissue, with a high proportion of IgG4+ plasma cells. We presented a rare case of IgG4-RSD with isolated involvement of the paranasal sinuses in the absence of multiorgan involvement. Methods A case report and comprehensive literature review. Results To our knowledge, only 11 cases of IgG4-RSD with paranasal sinus involvement have been reported. Patients with IgG4-RSD commonly present with epistaxis and symptoms that mimic chronic rhinosinusitis, e.g., rhinorrhea, nasal obstruction, and facial pressure. On imaging, an expansive and erosive process is described. Surgery provides tissue for immunohistologic evaluation; however, there is a paucity of evidence about the direct extent of surgical resection or medical therapies. Postoperative steroids were typically started, although the regimen was not standardized. Conclusion Few cases of paranasal sinus IgG4-RSD have been reported in the literature. Evidence-based recommendations regarding treatment and surveillance of paranasal sinus IgG4-RSD are lacking; however, most reports describe systemic steroids as the mainstay of treatment. This single subject analysis, with a review of previously reported cases adds to the expanding body of data related to this rare disorder.