Ewing sarcoma and primitive neuroectodermal tumors (ES/PNETs) are rare tumors that belong to a family of round-cell neuroectodermally derived tumors, and their optimal treatment remains a great challenge. This study presented a case of ES/PNET, arising in the esophagus of a 21-year-old female patient presented with progressive dysphagia. Computed tomography and endoscopic ultrasonography showed a well-defined, submucosal solid mass in the superthoracic esophagus. The accurate diagnosis after surgery was obtained through immunohistochemistry and genetic studies, namely the CD99 immunopositivity as well as the EWSR1/FLI1 gene rearrangement associated with t(11;22)(q24;q12) in tumor cells. The patient underwent localized tumor resection followed by chemotherapy and chest radiotherapy. The patient is doing well with no evidence of tumor recurrence or metastasis 18 months after surgery. Although the esophagus is a rare site for ES/pPNET, we can speculate that the treatment protocol of ES/pPNET should include multi-agent chemotherapy, surgery, and local radiotherapy in order to improve the prognosis based on our report.