Pulmonary alveolar microlithiasis (PAM) is a rare disorder of uncertain etiology and pathogenesis that is characterized by tiny calcium phosphate depositions in lung alveoli. Ectopic organ involvement has been reported infrequently. We hereby present a patient with PAM complicated by severe pleural calcification that has caused a constrictive cardiac physiology, a heretofore unknown occurrence/association. Based on our observations in this particular case, we suggest that a non-invasive echocardiographic examination may be well worth in the investigation of PAM patients in whom symptom onset at an early age has occurred, particularly if severe pleural calcification adjacent to the heart is present. Copyright © 2007 S. Karger AG.