Background <p>Type B aortic dissection associated with aortic coarctation is an extremely rare condition. We present a case that was treated successfully by endovascular repair.</p> Case presentation <p>A 46-year-old man was admitted to our hospital for severe chest pain radiating to his back. Contrast-enhanced computed tomography angiography revealed type B aortic dissection associated with coarctation at the distal end of the aortic arch. Combined treatment with angioplasty and endovascular stent-graft deployment was performed successfully. Three years later, contrast-enhanced computed tomography angiography showed that the coarctation had disappeared, with the residual dissection remaining asymptomatic.</p> Conclusion <p>For type B aortic dissection associated with aortic coarctation, endovascular repair seems to be an alternative to open surgery, especially for highly selected patients.</p>