Abstract
BACKGROUND: Ewing sarcoma is a rare malignant tumor that primarily affects children and young adults, commonly metastasizing to the lungs, bone, and bone marrow. CNS involvement is extremely rare, especially with the primary presentation of intracranial hemorrhage. Current protocols do not include routine neuroimaging, potentially delaying the detection of brain metastasis. Additionally, Ewing sarcoma patients are at risk of developing chemotherapy-induced thrombocytopenia that can significantly increase the possibility of intracranial hemorrhage. OBSERVATIONS: A 19-year-old male with metastatic Ewing sarcoma of the pelvis presented with sudden-onset severe headache, seizures, and unresponsiveness. Imaging revealed a large left frontoparietal intraparenchymal hemorrhage with midline shift requiring emergency surgical decompression. Histopathology confirmed metastatic Ewing sarcoma. Despite proper management, the patient's medical condition deteriorated, leading to subsequent death. LESSONS: Early and interval brain imaging should be considered in patients diagnosed with Ewing sarcoma, especially those undergoing chemotherapy with thrombocytopenic effects. Earlier detection of CNS involvement may facilitate timely intervention and improve outcomes. Future research should explore optimized surveillance strategies and the connection between metastatic intracranial Ewing sarcoma and the unusual presentation of hemorrhage. https://thejns.org/doi/10.3171/CASE25246.