<p style="text-align: justify;"><strong>Objectives:</strong> Extraosseous Ewing sarcoma is a variant of Ewing sarcoma that can occur anywhere in the body. There are few documented cases reported in the head and neck region and only two reported cases arising from the larynx.
<strong>Methods/Results:</strong> We report a case of a 45-year-old female who was found to have primary extraosseous Ewing sarcoma of the larynx. Initial workup of her new neck mass, appearing to arise from the thyroid gland, was concerning for lymphoma based on fine needle aspiration. However, further biopsy and immunohistochemistry confirmed a much different diagnosis. She received the current recommended therapy for this rare disease that has a high rate of recurrence.
<strong>Conclusions:</strong> These tumors can be mistaken for other neoplasms due to their primitive morphology and rare occurrence. We discuss the workup to allow for accurate diagnosis, treatment, and prognosis to improve morbidity and mortality from this rare disease.</p>