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Turner syndrome, also described as 45, X, may present with most serious cardiovascular anomalies including risk of aortic dissection and rupture. In emergency situation, management for aortic dissection with complicated anatomy accompanying vascular anomaly is challenging. Here, we report a rare case of ruptured type B aortic dissection with aberrant subclavian artery and partial anomalous pulmonary venous connection in a Turner syndrome. Through right carotid-subclavian artery bypass and thoracic endovascular aortic repair, successful hybrid endovascular management correlated with a favorable result in this emergency situation.