Abstract Background: We report the rare case of an adult with a primary extranodal polymorphous hemangioendothelioma at dorsum, treated with endovascular coiling followed by open surgery resection. Clinical history, findings of spinal arteriography, histopathological findings and combined surgical procedure are reported. Case report: A 22-year-old male patient complained of a dorsal mass that has been increasing in size, painful, soft, no mobile and without neurological symptoms. A vascular tumor was suspected and an endovascular followed by an open surgical approach was performed. Histopathological diagnosis of an extranodal polymorph hemangioendothelioma was documented and a total resection was confirmed by free-tumor resection margin. The postoperative course was uneventful. Conclusion: Polymorph hemangioendothelioma is a rare vascular tumor. given the high vascular features of the lesion and, therefore, the high rate of bleeding during surgery.