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Pulmonary alveolar microlithiasis is a rare, chronic lung disease with bilateral intra-alveolar calcium and phosphate deposition throughout the lung parenchyma. This is a case history of a 40-year-old female with a family history of lung disease, presenting with progressive respiratory symptoms for 5 years. She had clinical and radiological features of pulmonary alveolar microlithiasis and responded poorly to medical treatment, subsequently admitted with severe respiratory insufficiency, pulmonary hypertension and right heart failure. She died after repeated attempts at resuscitation. Her autopsy revealed atherosclerotic pulmonary arteries with intra alveolar deposits of concentrically laminated calcific bodies which confirmed the diagnosis of pulmonary alveolar microlithiasis. The disease evolved in a progressive protracted course with respiratory insufficiency which led to corpulmonale.