Introduction: Ewing sarcoma (ES) is a rare primary malignant tumor of the maxillofacial area. On the other hand, the primary involvement of the mandibular condyle is really very rare and any misdiagnosis may significantly affect the prognosis of the disease. Case Presentation: This report describes a 29-year-old female patient who has been treated for temporomandibular disorder before being referred to our department. The patient was admitted with a diagnosis of ES and managed by primary induction chemotherapy, followed by tumor resection, radiotherapy and postoperative chemotherapy. Conclusions: ES of the mandibular condyle is really rare. Therefore, early diagnosis and multimodal therapy are essential for having a better prognosis of these lesions.