We demonstrate first results of breath-hold 3D-UTE MRI for ventilation imaging in CF patients. Ten patients underwent 3D-UTE MRI of the lungs. Data were acquired in breath-hold for 5 different breathing states, in order to obtain information regarding the ventilation. 3D-UTE MRI provided sufficient morphologic image quality for the detection of structural lung disease and site-resolved information regarding pulmonary ventilation. Ventilation measurements correlated well with parameters of conventional lung function testing and were in accordance with pulmonary ventilation measured with techniques published recently. The technique could be an innovative method able to enhance diagnosis and monitoring of structural lung disease.