Aortic coarctation is often linked to bicuspid aortic valve and only rarely to congenital aneurysm of the circle of Willis. It is possible that a common pathogenetic substrate determines this association, but it has not yet been demonstrated. In this report we describe the case of a 32-year-old woman, affected by previous anamnestic functional cardiac murmur. She has suddenly presented a hemorrhagic stroke due to the breaking of the sylvian artery, angiographically demonstrated and surgically corrected. On the same occasion aortic angiography revealed aortic coarctation. Later, an echocardiographic exam demonstrated the coexistence of bicuspid aortic valve. Afterwards the patient underwent angioplasty in order to correct aortic coarctation. Any coexisting cardiac and vascular disease may influence survival in unoperated adults with aortic coarctation, which can even be lethal. In this case the simultaneous presence of these three malformations was particularly dangerous since the patient was not enough symptomatic and so an early diagnosis was not possible. Therefore, we strongly recommend to perform echocardiography in every young patient affected by hemorrhagic stroke and to take into consideration the possibility of performing cerebral angiography in all cases of aortic coarctation.