Résumé : BACKGROUND AND PURPOSE: In 1964, the first case of "de novo" aneurysm has been reported by Graf and Hamby. The risk of late formation of aneurysm is unknown. Some factors could influence risk: history of hypertension, oral contraceptives, cigarette smoking. We report a rare case of "de novo" aneurysm associated with Ehlers-Danlos syndrome. RESULTS: A 35-year-old female developed a subarachnoid hemorrhage (SAH). Angiography demonstrated 4 aneurysms. Five years before, she presented a SAH. At that time, four vessels angiography demonstrated only one PICA aneurysm. A Ehlers-Danlos syndrome was suspected, which was demonstrated on skin biopsies. CONCLUSION: At our knowledge, this is the first case of "de novo" aneurysm associated with a Ehlers-Danlos syndrome.