Pulmonary alveolar microlithiasis is a rare disease and only 32 cases
have been reported in children under 12 years of age. The first report
on Saudi children with this disorder and on affected cousins is prese
nted, supporting the possible hypothesis of it being an autosomal rece
ssive disorder. The importance of differentiating it from other condit
ions, particularly pulmonary tuberculosis, and the current approach to
diagnosis and management are discussed.