Soft tissue sarcomas are rare tumours of the connective tissue. The knowledge of this rare entity is necessary for an accurate diagnosis. The incidence in Europe is about 3,6/100 000 per year. The first line treatment consists of a wide resection of the tumour, possibly combined with an adjuvant radiation therapy. A chemotherapy can be indicated and has to be evaluated for each case individually. A biopsy should be done before any treatment and in accordance with the oncological resection approach. The management of the soft tissue sarcoma has to be carried out by a specialist sarcoma multidisciplinary team.