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A 13-year-old boy with primary ciliary dyskinesia (PCD) had progressive severe cystic bronchiectasis of the left middle and lower lobes on serial CT scans, in the context of situs inversus totalis and a trilobed left lung. He was chronically infected with Pseudomonas aeruginosa and Mycobacterium avium complex. Given the progressive nature of the bronchiectasis and declining clinical status, the decision was made to proceed to left middle and lower bilobectomy to prevent further destruction of the less diseased lung. To monitor outcomes, phase-resolved functional lung (PREFUL) MRI, spirometry and Lung Clearance Index (LCI) derived from multiple breath nitrogen washout (MBNW) were performed 1 week prior and 16 weeks after the bilobectomy. MRI examinations were performed on a 3T Siemens scanner during free breathing with no contrast agent administered. PREFUL processing was used to derive regional pulmonary ventilation and perfusion distribution from proton MRI data.1