BACKGROUND A cervical aortic arch (CAA) refers to a high-riding aortic arch (AA) that often extends above the level of the clavicle. This condition is very rare, with an incidence of less than 1/10000. METHODS A 29-year-old woman was admitted to the otolaryngology department of our hospital for repeated bilateral purulent nasal discharge for the prior 3 mo. The patient was diagnosed with chronic sinusitis and chronic rhinitis at admission. A preoperative noncontrast chest computed tomography scan showed a high-riding, tortuous AA extending to the mid-upper level of the first thoracic vertebra with local cystic dilatation. A further computed tomography angiography examination showed that the brachiocephalic trunk, left common carotid artery, left vertebral artery (LVA) (slender), and left subclavian artery sequentially branched off of the aorta from the proximal end to the distal end of the AA. The proximal end of the right subclavian artery (RSCA) was tortuous and dilated. The AA showed tumor-like local expansion, with a maximum diameter of approximately 4 cm. After consultation with the department of cardiac macrovascular surgery, the patient was diagnosed with left CAA with aneurysm formation and an anomalous RSCA and LVA and was transferred to that department. The patient underwent AA aneurysm resection and artificial blood vessel replacement under general anesthesia and cardiopulmonary bypass. No abnormality was found during the 2-mo follow-up after discharge. CONCLUSIONS A CAA is a rare congenital anomaly of vascular development. The present unique case of CAA with aneurysm formation and an anomalous RSCA and LVA enriches existing CAA data.