Skip lesion is not uncommon feature in osteosarcoma and considered to be importantly associated with poor prognosis factor, and thus, should be excised with the main mass. The accurate pre-operative evaluation of the intramedullary extent of osteosarcoma is essential, because it determines the level of bone resection. Among the reliable detection methods, bone scan has a drawback of high rate of false negative results and regional MRI has a difficulty to cover the whole involved lesions without clinical suspicion. The authors report a case of osteosarcoma of the distal femur with a proximal skip lesion that was not detected by either regional MR imaging or by bone scan, but which was visualized by FDG-PET/CT.