IntroductionEpithelioid hemangioendothelioma (EHE) is a rare vascular tumour that originates from vascular endothelial or pre-endothelial cells. Its demographic characteristics are unclear and there is no racial and sex predilection,8The overall incidence of EHE is 0.230 per 1000000 person-years.8 EHE in general affects patients in the age range 16-74 years but EHE of the bone is more prevalent in the third and fourth decade of life.2,3,5EHE can develop in any part of the body but is reported to more frequently in the liver (34%), lungs (21%), bone (19%) and other sites (26%). 1.,2 It was first described and named in 1982 by Weiz and Enzinger and is classified in the 2020 WHO classification of bone tumours as a low-to-intermediate grade neoplasm.2.,3 Malignant vascular bone tumours are rare and constitute less than 1% of primary bone tumours.3 Bone involvement usually involves the lower extremity (62%), upper extremities (14%) and vertebra (10%)3. As such primary EHE of the spine is very uncommon, with the thoracic spine most likely to be involved followed by cervical, lumbar, and sacral levels in order of decreasing frequency3.Here we report on a 26-year-old female with thoracic T2 and T3 lesions in keeping with primary EHE of the spine, her clinical presentation, work up including laboratory, immunohistopathological and imaging studies. We also conclude on her management and current condition. There are currently no prior clinical case reports on primary EHE of the spine in South Africa or Africa as a whole.