Swyer-James-MacLeod syndrome (SJMS) is considered to be a relatively uncommon disease presenting with unilateral hyperlucent lung due to hypoplasia of a pulmonary artery and bronchiectasis of the affected lung. In this report, we describe the ventilation-perfusion (V/Q) scan findings of nine male recruits (aged 20-29 years, mean 24.4+/-2.96 years) with SJMS in whom the diagnosis was first established in adulthood. V/Q scan findings of all patients were compared with those on planar radiographs, pulmonary function studies, high resolution computed tomography (HRCT) and digital subtraction angiography (DSA). The ventilation (133Xe) and perfusion (99Tcm-macro-aggregated albumin) scans showed the characteristic pattern of a matched V/Q defect and marked air trapping on the washout phase on 133Xe scintigraphy. HRCT displayed hypodense lung with integrity of main airways, and markedly diminished vasculature on the affected side in all patients. A smaller pulmonary artery on the affected side with poor peripheral vasculature was observed with DSA in all patients. All patients had features of obstructive airway disease in varying degrees on pulmonary function studies. In contrast to other imaging methods, bronchiectasis as an etiological factor was displayed on HRCT. Some pulmonary areas, which were normal on HRCT and planar radiographs, showed air trapping on V/Q scan. Although a V/Q scan was more helpful in determining the extent of the disease and correlates well with conventional imaging methods, HRCT was the most valuable imaging method for the evaluation of aetiology in unilateral hyperlucent lung.