Introduction: Ewing's sarcoma is a primary malignant bone tumor that affects children and young adults. In rare cases, it affects the bones of the face. The diagnosis is difficult because signs are non-specific. Positive diagnosis is based on a cytogenetic examination showing a specific translocation of the EWS gene. Treatment involves a sequence including chemotherapy, oncological surgery and radiotherapy. Observation: The case presented is that of a 4-year old girl initially sent for suspicion of dental cellulitis. On clinical examination, she had recent mandibular dental mobility and a gingival mucosa swelling. Signs of bone lysis interesting the mandibular symphysis was observed on the orthopantomogram. She had no pain or symptoms indicating neoplasia. This atypical situation led to a bone biopsy who revealed the diagnostic of mandibular Ewing's sarcoma. A suitable medical care in a specialized unit was able to be started without delay. Discussion: The literature reports other cases also wrongly diagnosed as an infectious pathology of dental origin. Conclusion: This case shows that the diagnosis of mandibular Ewing's sarcoma must be evoked in front of any atypical mandibular lesion of the child, regardless of his age or sex. Physicians and dental surgeons specialized in oral surgery are then in the foreground to help establish early diagnosis and ensure rapid management by limiting the functional and aesthetic sequelae related to localization.
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