Atypical presentation of Ewing's sarcoma with a single left orbital metastasis

Summary Background We present an uncommon case of Ewing’s sarcoma in a 16-year-old boy. Case Report This case can be considered unique because of the atypical presentation, normal laboratory tests and absence of the typical symptoms such as pain, masses or swelling, fatigue or weight loss, breathing problems linked to lung metastases or pathologic fractures. The only event that brought the patient to our attention was the sudden onset of left proptosis. Conclusions The final histopathology together with CT and PET-CT findings led to the diagnosis of a multi-metastatic Ewing’s sarcoma involving the orbit, skeleton, bone marrow and lymph nodes.