Spontaneous (idiopathic) thoracic aortic rupture (STAR) is uncommon and assumes a rupture of the normal-sized thoracic aorta with no visually apparent aortic disease. Since 1961 about fifty reports have been published. STAR is established in cases of thoracic normal-sized aorta rupture with no traumas, aneurysms/dissections, infection, inflammation, connective tissue diseases, aortic and adjacent organs tumors/metastases, previous surgery, and occurring during pregnancy and the peripartum. Atherosclerosis penetrated atherosclerotic ulcer, neurofibromatosis type I, peri- and postpartum estrogen-mediated elastin irregularities of the aortic media, and cystic medial necrosis (CMN) were identified as a cause of STAR when histopathological examinations were performed. A case of a 68-year-old man with giant STAR of the descending aorta in the background of CMN is reported here. The patient in terminal hemodynamic condition was successfully treated by delayed open surgery two weeks after the disease's onset.
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