혈관형 엘러스-단로스 증후군은 취약한 혈관으로 인해 주로 출혈성 합병증으로 나타나지만, 저자들은 동맥경화 등의 위험 인자가 없어 보이는 젊은 남성에서 신동맥 박리 및 혈전성 신장 경색, 다발성 혈관 기형, 입원 후 발견된 복강 내 출혈 및 무증상의 혈흉, 결체조직의 이상 증상 등으로 유전자 검사를 실시하여 혈관형 엘러스-단로스 증후군 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.Renal infarction is a condition caused by sudden disturbance of renal arterial blood flow, which occurs frequently in cardioembolic disease, renal artery injury (trauma, Marfan syndrome, rarely Ehlers-Danlos syndrome), and in association with a hypercoagulable state. Ehlers-Danlos syndrome is a rare hereditary connective tissue disease characterized by skin fragility, hyperelasticity, hypermobility of small joints, and easy bruising. Among the subtypes, vascular Ehlers-Danlos syndrome is a very rare genetic disease caused by a mutation in the COL3A1 gene. As blood vessels are fragile due dysfunctional collagen synthesis, complications of bleeding due to arterial dissection or rupture are common in patients with this disease. Here, we report a case of vascular Ehlers-Danlos syndrome diagnosed based on a renal infarction caused by thrombus and renal artery injury; we also present a review of the relevant literature.