A 29-year-old male presented with a 6-month history of left hip pain. Radiography and CT demonstrated a 6-cm lytic lesion of the left acetabulum. F-18 FDG PET for staging of the biopsy-proven malignant PEComa demonstrated intense hypermetabolism corresponding to the lytic left acetabulum. The patient underwent left hemipelvectomy with reconstructive surgery. On a follow-up F-18 FDG PET scan 3 months after initial surgery, a left lung pleural-based 9-mm nodule with hypermetabolism (SUVmax 4.1) was discovered and pathologically proven to be metastases from the primary osseous PEComa.