Chronic cough is a common chief complaint in ambulatory clinics. Unlike most cases that are caused by upper airway cough syndrome, gastroesophageal reflux disease, asthma, and non-asthmatic eosinophilic bronchitis, chronic cough can also be the presenting feature of a Chiari malformation. Our case is that of a 39-year-old female who had a chronic cough associated with shortness of breath, and when severe, associated with loss of consciousness. Her cough was refractory to conventional management. Further workup including pulmonary functions tests (PFT), laryngoscopy, high-resolution CT of the chest, an upper GI series, and esophageal pH manometry study were all normal. An MRI of her brain was obtained due to her syncopal episodes and revealed findings concerning a type 1 Chiari malformation. She subsequently underwent a Chiari decompression with patchy duraplasty and tonsilloplasty with cervical vertebrae 1 and 2 (C1-C2) laminectomy with a resolution of her symptoms. Chiari malformations are sometimes inherited but are often sporadic in nature, and, thus, appropriate diagnosis is key. Our patient is unique in that she presented at an older age, suggesting that atypical etiologies of a chronic cough refractory to conventional treatments must be considered.