The correlation between oesophageal dysfunction (OD), pathologic gastro-oesophageal reflux (GOR), and severity of pulmonary disease was studied in 12 patients with cystic fibrosis (CF). They were interviewed about symptoms of OD and underwent 24-h pH recording in the oesophagus, oesophageal manometry combined with reflux provocation tests, the acid perfusion test, the acid clearance test, lung function tests, and scoring of the chest radiograph. Six of the 12 patients reported symptoms of OD. Abnormal GOR, as shown by 24-h pH monitoring of the oesophagus, was found in eight of them. Altogether 9 of the 12 participants had at least one pathologic oesophagus test result. Results of radiologic examinations of the oesophagus, performed in six patients, were pathologic. The four patients with the best chest radiograph scores and the best lung function had significantly less signs and symptoms of OD and GOR than the other eight patients. We conclude that OD, GOR, and pulmonary disease covariate in CF.