Background: Loeys-Dietz syndrome (LDS) is a rare congenital connective tissue disorder (CTD) caused by mutations in the gene encoding for transforming growth factor-β receptors I and II. This recently described syndrome is characterized by aortic aneurysms and dissections, arterial tortuosity, and spontaneous organ perforation. The technical feasibility of endovascular interventions, particularly endovascular aneurysm repair (EVAR), in CTDs is relatively unknown. Methods and Results: A 38-year-old man presented with asymptomatic bilateral common iliac artery aneurysms measuring 5.3 cm on the right and 4.3 cm on the left. The patient had an extensive surgical and medical history, including a recently repaired Stanford type-A aortic dissection, total colectomy with end ileostomy for a colonic perforation, splenectomy for rupture, and cirrhosis secondary to chronic hepatitis C. The patient's CTD, multiple abdominal surgeries performed in the past, and ileostomy made him a poor candidate for open repair. We elected to offer him a complex endovascular repair and hoped to preserve his pelvic circulation by using "double-barrel" configuration of stent-grafts in the right iliac artery system. Successful deployment of the devices and repair of femoral access allowed routine discharge on postoperative day 2. At 6-month follow-up, the patient's pelvic circulation has been maintained, the aneurysms are excluded without endoleak, and sac regression has been shown. Conclusion: LDS is a rare connective tissue disorder characterized by vascular aneurysms and arterial tortuosity. When vascular reconstruction is necessary, open techniques are often preferred given the lack of data on endovascular procedures. In the present case, we report the first successful abdominal EVAR in a high-risk patient with LDS, providing excellent short-term results.