Juvenile xanthogranuloma, a rare type of non-Langerhans cell histiocytosis, generally manifests as widespread skin lesions, which is often self-limited. However, when other organs are involved, its outcome can be unfavorable, and there is no clearly defined consensus regarding what is the best imaging modality in monitoring the therapy. We report here findings of a series of FDG PET/CT scans during the course of clofarabine therapy in a 12-year-old girl with extracutaneous juvenile xanthogranuloma.