Primary tumors of the aorta are extremely rare and have been associated with aortic dissection, being the intimal sarcoma very aggressive with a propensity to metastasize early. Once the diagnosis has been confirmed, surgical resection may be curative, however the prognosis remains dismal, with death resulting in most patients. We describe a case of type A aortic dissection, with a intramural hematoma, associated with intimal sarcoma, in a young patient, presenting with a cerebral hematoma, ultimately resulting in the patient's death.