OBJECTIVE AND IMPORTANCEIntravascular papillary endothelial hyperplasia (Masson’s vegetant hemangioendothelioma) is a rare condition affecting the neuraxis. In the literature, only one case of this lesion involving the vertebral canal with spinal cord compression has been reported. We present a case of cauda equina compression due to this abnormality.CLINICAL PRESENTATIONA 17-year-old boy was admitted at our hospital with pain, numbness, paresis of the left lower extremity, and bladder dysfunction of approximately 1 month’s duration. Computed tomography and magnetic resonance imaging of the spine revealed a tumor within the spinal canal at the T12–L1 level.INTERVENTIONThe patient underwent a T12–L1 laminectomy. An epidural red nodular tumor was visualized and totally resected. The findings of the pathological examination were compatible with intravascular papillary endothelial hyperplasia. At follow-up examination 1 month after the operation, the patient had complete resolution of the pain, and the motor deficit and bladder dysfunction had improved significantly.CONCLUSIONThis rare benign vascular lesion may be clinically and histopathologically mistaken for an angiosarcoma. Because the intravascular papillary endothelial hyperplasia can be cured by complete surgical resection, it is important to distinguish between these two lesions to avoid inappropriate aggressive treatment.