The clinical data of a patient with primary extraskeletal Ewing’s sarcoma of the larynx were retrospectively analyzed, and relevant literature was reviewed. An 11-year-old boy presented with acute respiratory distress, underwent emergency tracheotomy and laryngeal mass biopsy, and the diagnosis of laryngeal Ewing sarcoma was confirmed by immunohistochemistry and molecular pathology. Recurrence of tumors was considered, and the treatment was a combination of radiotherapy and chemotherapy, with no recurrence or metastasis at 6-month follow-up. The extraskeletal Ewing’s sarcoma, which is rare arising from the larynx, lacks specificity in clinical presentation and early diagnosis is difficult. Individualized comprehensive treatment should be emphasized. The disease is prone to recurrence and metastasis, resulting in poor prognosis.

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