A 56-year-old female nonsmoker presented with episodic hemoptysis, without any other associated respiratory symptoms. Her medical history was notable for polycythemia rubra vera with portal vein thrombosis, which was treated with warfarin, but was complicated by portal hypertension. Esophageal varices were controlled by endoscopic band ligation. Chest radiograph and 64-slice computed tomography scanning failed to identify a culprit lesion. Bronchoscopy identified a vascular structure in the proximal trachea. A contrast-enhanced 320-multidetector row computed tomography scan of the neck showed a tortuous vascular channel in the trachea, which changed in appearance over time, consistent with a tracheal varix. The patient was changed to aspirin therapy and was evaluated by a cardiothoracic surgeon. A conservative approach was adopted, and the patient has had no recurrence of symptoms.