Epithelioid haemangioendothelioma (EHE), first described in 1982, is an uncommon malignancy comprising of around 1% of all vascular tumors and accurate diagnosis relies on specialist review of histology. Cervical spinal primaries are very rare and standard treatment for localised disease is surgery. Data regarding management of localized but unresectable tumors are limited. We undertook a literature review in order to highlight its clinico-pathological features and we describe a first case of an unresectable cervical EHE, successfully treated with radical radiotherapy.