This case report presents a 34-year-old male with Ehlers–Danlos syndrome, type 2 diabetes mellitus, aortic valve regurgitation, and aortic bulb aneurysm. Following spine surgery for thoracic–lumbar stabilization, the patient underwent assessment for aortic bulb aneurysm and aortic valve replacement surgeries. Five months post spinal surgery, a coronary computed tomography angiography was performed. The coronary computed tomography angiography revealed unique findings, including the absence of the left main coronary artery, right coronary artery dominance, ectopic origin of the left circumflex artery from the right sinus of the valsalva, a coronary–pulmonary arterial fistula originating from the right sinus of the valsalva, and an additional right pulmonary vein. The patient was qualified for surgical treatment for an aortic bulb aneurysm, was informed about the high surgical risk, and is awaiting surgery. This case underscores the rarity of Ehlers–Danlos syndrome coexisting with multiple coronary artery anomalies. The presence of a coronary–pulmonary arterial fistula further emphasizes the need for specialized patient monitoring when Ehlers–Danlos syndrome and coronary anomalies converge.