Hemangioendotelioma is a group of rare vascular tumors with border-line behavior. They may involve internal organs, limbs, bones and soft tissue. The histological variant epitheliod hemangiothelioma is locally aggressive and has metastatic potential. We present a case of a 41-years old woman affected by epitheliod hemangiothelioma of the right quadriceps. The patient complained of localized pain in the anterior region of the right thigh, which started about 3 months earlier during physical exercise. For the increasing size of the lesion, the patient was referred to our Department. On physical examination, the lesion appeared elastic, fixed to the surrounding tissues and painful under pressure. An ultrasound examination revealed a small avascular lesion and a MRI examination showed an oval formation measuring about 18 mm in diameter of nonspecific significance. A surgical removal of the lesion was performed and diagnosis of epithelioid hemangiothelioma was achieved. A complete removal of the quadriceps muscle was then accomplished. In this paper, clinical and oncologic aspects of this rare tumor are discussed KEY WORDS: Epithelioid hemangiothelioma, Lower limbs tumor, Rare tumors.

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