Metastatic central nervous system (CNS) involvement is rare in pediatric primary extracranial Ewing sarcoma (ES). We describe the incidence and course of six patients with extracranial ES who developed metastatic CNS lesions treated at a single institution. Median time to CNS disease detection was 16.3 months (10.0-28.3 months). Event free and overall survival after CNS disease detection were 1.9 months (0.4-10.3 months) and 4.6 months (1.1-50.9 months), respectively. One patient was alive at the time of analysis. Clinical status and ability to obtain disease control should be considered when making decisions regarding aggressive interventions in these patients with poor prognosis.