Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour that can occur in a wide variety of anatomical sites including the liver, lungs, skin, bone and is rarely reported in the oral cavity. The condition is classified as a tumour of intermediate malignancy as, in contrast to other types of haemangioendothelioma, EHE can result in distant metastasis and the death of the patient. Due to the minimal number of cases reported of individuals diagnosed with EHE, no definitive data on different treatment options exist and complete excision with follow up and consideration of adjuvant radiotherapy is the universal treatment option. This case report highlights the investigation, diagnosis and treatment of a 25-year-old man who was diagnosed with EHE arising within the mandible. The article also presents the published literature of documented cases of mandibular and maxillary EHE.