We report the case of a 38-year-old man with Loeys-Dietz syndrome (LDS) who presented in hemorrhagic shock due to rupture of a right internal iliac artery aneurysm, with contrast-enhanced computed tomography (CT) also revealing multiple aneurysms involving the aortic root, abdominal aorta, and several visceral and pelvic arteries. Because of hemodynamic instability and a history of prior abdominal surgery, open surgical repair was considered high-risk, and emergency endovascular treatment was chosen instead. Coil embolization was performed at the proximal neck of the internal iliac artery, and the distal branch vessel was embolized with a mixture of n-butyl-2-cyanoacrylate (NBCA) and Lipiodol, achieving complete hemostasis without unintended embolization. The patient's hemodynamic status stabilized, and he was discharged ambulatory on postprocedure day 30. Genetic testing subsequently confirmed a missense mutation in transforming growth factor-beta receptor type 1 (TGFBR1), establishing the diagnosis of LDS. This case illustrates that combined NBCA and coil embolization can be an effective and rapid hemostatic strategy for a ruptured internal iliac artery aneurysm in patients with LDS, for whom open repair carries substantial risk.