Pulmonary alveolar microlithiasis (P.A.M.) is a rare pulmonary disorder that pursues usually an asymptomatic course and can culminate in severe respiratory failure. We report a 48 year old Saudi female patient with P.A.M. who deteriorated rather steadily after the initial 18 years of asymptomatic course until a frank type I respiratory failure is established. Single lung transplantation (S.L.T.) was performed successfully and the patient returned to full daily activity and has now survived 12 months post S.L.T. The immunosuppression consisted of Cyclosporine-A 10 mg/kg/day, azathioprine (immuran) 2 mg/kg/day and prednisolone 10 mg daily. The bronchial anastomosis was done by telescoping the recipient and donor main bronchus without omental wrap. A significant bronchial stricture of the anastomotic site occurred 4 months post S.L.T. which was dilated endoscopically with good clinical and bronchoscopic result. No episodes of rejection or infection were encountered so far.