Extraskeletal Ewing's sarcoma (EES) is a rare aggressive malignant tumor, usually occurring in the chest wall of children and young adults. Although EES shows aggressive growth pattern, it seldom demonstrates distant metastasis at diagnosis. Herein, we present computed tomography (CT) and positron emission tomography computed tomography (PET/CT) findings of EES in anterior mediastinum in a 68-year-old man, showing multi-organ distant metastasis at diagnosis. It is another atypical case with unusual presentation in point of old age, mediastinal location and distant metastasis at diagnosis, showing PET/CT findings.