Background: Pulmonary alveolar microlithiasis (PAM) is an inherited lung disease in which calcium phosphate deposits (chalcosphere) build up in the distal alveoli. There is no medical treatment for PAM, for patients with end-stage disease, lung transplantation is an option. Due to PAM is a rare condition with less than 1 per million prevalence, it becomes an exceptional case during pregnancy. We reported a pregnant PAM case and reviewed with other six cases in the literature to date. Case Presentation: A 21-year-old nulliparous pregnant woman with PAM presented in this report. The patient’s obstetric and respiratory functions follow-up were unremarkable until 35 weeks of her gestation. Because of the patient had shortness of breath at 35 weeks of gestation, she was evaluated and hospitalized. She delivered by cesarean section at 38th gestational week. Conclusion: It is still unknown that pregnancy how affect the women with PAM because the disease is rare. We reported a pregnant PAM case and reviewed with other six cases in the literature to date.

Keywords: Calsinosis, lung disease, microliths and micronodular opacities, pregnancy, pulmonary alveolar microlithiasis, perinatal outcome