Immunoglobulin G4 (IgG4)-related disease is an inflammatory condition characterized by IgG4 positive plasma cell infiltration. It can affect any organ in the body and mainly involves the pancreas, liver, biliary tracts, orbits, salivary glands and lymph nodes. It can manifest as an inflammatory pseudotumor. Pseudolymphoma as an inflammatory pseudotumor is a group of benign tumors that exhibit histological and clinical features suggestive of malignant lymphoma. Studies on IgG4-related disease are rarely reported, and no case of the disease that involved the maxillary bone and adjacent soft tissue, except for the skin, has been reported. Therefore, we report herein a case of pseudolymphomatous IgG4-related disease that involved the maxilla, with a literature review.