Introduction. - Pulmonary alveolar microlithiasis is a rare disease characterised by the formation and deposition of calcium phosphate microtiths in the lung. It is an autosomat recessive disorder, for which mutation in the SLC34A2 gene was recently found to be responsible for the disease. Observations. - We report on four cases of pulmonary alveolar microlithiasis. Three patients were asymptomatic. The diagnosis was made after histological confirmation in three patients. The outcome was marked by the death of one patient. Conclusion. - Pulmonary alveolar microlithiasis is a rare disease. Diagnosis is made with high-resolution computed tomography, which exhibits the calcic character and distribution of the lesions, thus avoiding the need to perform lung biopsy. We suggest that a literature review be performed. (C) 2008 Publie par Elsevier Masson SAS.