Twenty-five children aged 8 months to 17 years were studied to determine the usefulness of computer-assisted ventilation-perfusion(V–P) scanning in analysis of regional pulmonary function in cystic fibrosis. Ventilation and perfusion scintiphotographs were compared with the patients' radiographs. Regional fractional exchanges of air and relative regional V–P ratios were obtained. Whole-lung air exchange was significantly correlated with standard pulmonary function tests and radiograph scores. Ventilation-perfusion ratio gradients were reversed in patients with predominantly upper zone disease, and regional V–P ratios were uneven in distribution. Quantitative assessment of regional ventilation and perfusion is a useful technique for assessing severity of pulmonary disease in children with cystic fibrosis.