We report here the case of an epithelioid haemangioendothelioma (EHE) arising in the nasal cavity which is, to the best of our knowledge, the first ever described example in the world literature in that particular site.

The patient is a 23-year-old male who presented with repeated episodes of epistaxis from the nasal cavity and with a 1.5 cm reddish, polypoid, smooth, spontaneously bleeding nodule in the rightmiddle meatus. This lesion was histologically diagnosed as epithelioid haemangioendothelioma. Immunohistochemically the neoplasm displayed striking positivity for CD31, CD34 and vimentin. A surgical approach was performed by ’facial degloving’, removing the right inferior turbinate, the anterior two-thirds of the middle turbinate and the medial wall of the ethmoid bone. After 12 months follow-up the patient is disease-free, without any local or distant recurrence.