Ewing sarcoma is children’s second most common malignant bone tumor after osteosarcoma. Relapsed and/or treatment-refractory cases have a poor prognosis. Cabozantinib is a tyrosine kinase inhibitor (TKI) that targets vascular endothelial growth factor (VEGF) and MET. Here, we present data on two children with metastatic Ewing sarcoma who received cabozantinib therapy. In both cases, the disease regressed significantly, symptoms improved, and side effects were manageable. Further research is needed to determine whether cabozantinib can maintain sustained disease control in children with Ewing sarcoma.