Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells. The presentation of IgG4-RD is heterogenous, making it difficult to diagnose. IgG4-RD presenting as a parapharyngeal mass is extremely rare. This report discusses the case of a 69-year-old African American female presenting with intermittent bilateral frontal headaches. Initial imaging revealed an ill-defined parapharyngeal mass encasing the left internal carotid artery and left internal jugular vein. Subsequent biopsy and immunohistochemistry showed a high concentration of IgG4-positive plasma cells with storiform fibrosis, despite normal serum IgG4 levels. The patient opted for conservative management. The localized parapharyngeal mass has remained stable over two years on annual imaging. This case report highlights that IgG4-RD can have varied and nonspecific presentations requiring high clinical suspicion to diagnose. Histopathology and IgG4 staining are vital to confirm the diagnosis of IgG4-RD, particularly in atypical cases not meeting the standard inclusion criteria.