Soft tissue sarcomas are a group of rare solid tumors with distinct clinical and pathological features. They arise from mesenchymal tissue with heterogeneous differentiation. They account for less than 1% of all malignant tumors. Sarcomas of the great vessels usually present in the aorta, pulmonary artery, and inferior vena cava. Peripheral arterial sarcomas are exceptionally rare. They have been reported in the iliac and common or deep femoral arteries and are frequently undifferentiated. The majority of peripheral arterial sarcomas are leiomyosarcomas or angiosarcomas. Primary neoplasms of the major blood vessels are divided into three categories based on their site of origin: large veins, pulmonary artery, aorta artery, and its branches. In the following report we present the case of a male in his thirties with a history of Hodgkin’s lymphoma, treated with first- and second-line chemotherapy regimens, who achieved a complete response after treatment, with no evidence of disease. Then in 2014 he was diagnosed with intimal sarcoma of the basilic vein in the left arm and treated with surgery as first line of treatment followed by adjuvant chemotherapy and radiation therapy. He later presented with lung metastases so metastasectomy was performed. The patient is currently being followed. The purpose of this paper is to present the case as there are not many reports on cases of this type and the site of presentation is unusual. The diagnostic approach is also described. Patients who receive radiation therapy as part of treatment for lymphoma are predisposed to develop sarcomas, which was not the case in this patient, so that this association may be explained by the presence of a genetic syndrome.