Lymphomatoid granulomatosis is an Epstein- Barr virus-positive large B cell lymphoma. Extranodal disease is common, involving the lung, kidney, liver, brain, and skin. We present a case of an 86-year-old male who was admitted due to fever and cough. Several reddish nodules were noted on the skin and biopsy was done. The pathological findings revealed lymphomatoid granulomatosis. He received rituximab for treatment. A sequence Serial examination of PET/CT was done as disease staging and treatment monitoring during the course of the disease, displaying an initial remission achieved after the treatment, but a dramatic relapse shortly afterwards. It is obvious that the surveillance in our case is insufficient merely according to the results of FDG PET/CT. Although PET/CT is an excellent tool for disease surveillance in classical lymphoma, but its role in lymphomatoid granulomatosis is unclear.