The oral, pharyngeal, and esophageal stages of swallowing were evaluated in 8 patients with recessively or dominantly inherited pure sensory ataxia. Six patients had swallowing difficulties: solid bolus obstruction, coughs during eating, and choking episodes. One patient had chronic bronchitis and another had recurrent pneumonia. The patients underwent a biphasic radiological barium swallow, including videofluoroscopy. No patient had a completely normal swallow. All had normal oral function, whereas pharyngeal function was abnormal in 6 patients. Esophageal function was abnormal in 6 patients. The swallowing dysfunction did not correlate with the severity of motor or sensory dysfunction in the limbs, nor with age or duration of ataxia. Our study shows that swallowing dysfunction is common in hereditary sensory ataxia. This dysfunction is likely to be due to involvement of the nucleus of the solitary tract in the brainstem. Despite some of the patients having suffered from choking episodes and others from bronchopulmonary complications, they did not spontaneously admit dysphagia. Swallowing should be evaluated thoroughly in patients with hereditary sensory ataxia since dysphagia in these patients might bring serious and potentially fatal complications.