Ewing's sarcoma is part of a rare group of malignant neoplasms that are localized frequently in the long bone of the lower extremities, the humerus, and the pelvis. However, Ewing's sarcoma can also arise from the rib. We described a 45-year-old male with Ewing's sarcoma of the left first rib, who presented with tenderness and numbness in the upper back and left upper limb for 2 months. CT, MRI and PET were performed and a tumor mass about 2.8 cm at the left first rib was found. Ewing's sarcoma was confirmed by biopsy. He received neoadjuvant chemotherapy, and then tumor-wide excision was performed via the anterior approach. We present this case and discuss its rare presentation, and also provide a literature review.