We present a patient with high $^{18}$F-fluorodeoxyglucose (FDG) uptake detected in a neurofibroma that was confused with sarcomatous transformation on a positron emission tomography/computed tomography (PET/CT) scan. A 39-year-old male patient with a 20-year history of neurofibromatosis-1 (NF-1) performed FDG PET/CT scan for the evaluation of lesions with sarcomatous transformation. The FDG PET/CT images demonstrated varying degrees of increased FDG uptake in the multiple nodules throughout whole body. The left pelvic mass with the highest FDG uptake had a maximum standardized uptake values (maxSUV) 5.0 and surgical resection was performed. Histological analysis confirmed the presence of a benign neurofibroma infiltrated with inflammatory cells.