Lymphomatoid granulomatosis is a rare Epstein-Barr virus-related lymphoproliferative disorder. We describe a case of a 42-year-old female with lupus nephritis and immunosuppression post renal transplant, who was diagnosed with central nervous system and lung lymphomatoid granulomatosis, as well as an Epstein-Barr virus-positive oesophageal ulcer, and was staged and followed up long term with multiple 18F-fludeoxyglucose positron emission tomography/CT scans and brain MRIs after achieving a complete metabolic response with rituximab.