During the course of evaluation and management of neck masses, consideration for Immunoglobulin G4-related disease (IgG4-RD) should be given. IgG4-RD is relatively a new growing entity of immune-mediated origin, characterized by a mass-forming lesion, the infiltration of IgG4-positive plasma cells and occasionally elevated serum IgG4. The most common manifestations are parotid and lacrimal swelling, lymphadenopathy and autoimmune pancreatitis.

A previously healthy 72-year-old man was referred to our clinic with a 2-month history of left cervical lymph node enlargement without systemic manifestations . A cervical lymph node biopsy was planned because of elevated serum IgG4 levels. Pathological findings showed prominent infiltration of IgG4-postive plasma cells in the lymph node. After steroid therapy, a computed tomography scan revealed a decrease in the cervical lymph node size.

This case illustrates the importance of including IgG4-RD in the differential diagnosis of a cervical lymph node enlargement.