Wilson-Mikity syndrome (WMS), an uncommon cause of respiratory distress presenting after birth, is radiologically characterised by varying degrees of interstitial thickening and bilateral cyst-like foci of hyperinflation. Aetiology and pathogenesis are still unknown. There are few reports of WMS in the paediatric literature and none describing the features and value of high-resolution CT. The purpose of this report is to describe the radiographic findings and high-resolution CT appearance of WMS and to correlate them with the histopathological findings.