OBJECTIVES: To  present  an  uncommon  cause  for  a  submandibular  mass  and  review  of  the literature.
METHODS:
Design: Case Report
Setting: Tertiary Government Hospital
Patient: One
RESULTS: A  25-year-old  lady  presented  with  a  painless  chronic  submandibular  swelling. Ultrasound identified a solid mass following which an uncomplicated core biopsy was performed obtaining    an    accurate    pre-operative    histopathological    diagnosis.    Pre-operative    arterial embolization of this vascular mass led to a relatively bloodless wide local excision. Radiological imaging for distant metastases was negative.
CONCLUSION: Epitheloid  Hemangioendothelioma  is  an  uncommon  cause  for  a  submandibular mass.   A malignant   vascular   soft   tissue   tumor   with   morphologic   characteristics   similar   to carcinomas, melanomas and epitheloid sarcomas, it has a high rate of metastasis and morbidity when it affects the soft tissues and viscera. Immunohistochemistry provides clues to differentiation and recommended treatment consists of a surgical wide local excision with regional lymph node resection. As there are no established standard therapeutic protocols for this disease due to its rarity, an individual case-by-case approach and follow-up needs to be undertaken.