Clear-cell sarcoma (CCS) is a rare, malignant, soft-tissue tumor, which involves the extremities, particularly the foot and foot joint tendons and aponeuroses. It is morphologically similar to but histochemically distinct from malignant melanoma. CCS arising in the gastrointestinal tract has rarely been reported. The prognosis of CCS is reportedly poor because of the high incidence of metastases at the time of initial diagnosis and the high frequency of recurrence. We report a case of early-stage CCS of the small intestine detected by ¹⁸F-fluoro-2-deoxy D-glucose Positron Emission Tomography/Computed Tomography (FDG-PET/CT) during the comprehensive examination of an inflammatory reaction. In this case, FDG-PET/CT clearly visualized the lesion, which was difficult to detect by contrast CT. J. Med. Invest. 56: 70-75, February, 2009