Lymphomatoid granulomatosis (LYG) is an uncommon Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder distinct from lymphoma. LYG primarily occurs in the lung with or without accompanied lesions in the skin, central nervous system, kidney, gastrointestinal tract, nose, eyes, liver and oral cavity. Solitary extrapulmonary LYG is extremely rare, and whether solitary lesions progress onto pulmonary development and dysfunction is controversial. Herein, we report a case on a solitary LYG in the maxilla gingiva with bone exposure in a patient who had been taking methotrexate for rheumatoid arthritis.