Aortic malignancies are rare diseases, of which only about 200 cases have been described in the literature. Most common forms are high-grade sarcomas, in over 80% of patients, which predominantly grow intimately and which can occur at any location in the aorta. In addition to non-specific symptoms, such as fever, hypertension and general weakness, aortic sarcomas in one third of patients manifest as peripheral embolism or, in the case of mural sarcoma, as an aneurysm or aortic rupture. In diagnostic testing, MRI is considered the gold standard, as CT angiography or PET CT can only show non-specific signs. Since aortic malignancies often manifest themselves as other diseases, diagnosis and therapy are often delayed. In therapy, the focus is on radial tumour resection with aortic replacement, possibly followed by adjuvant medical therapy. The 1-, 3- and 5-year survival rates are 26%, 7.6% and 3.5%, respectively.