Scant literature is available regarding pregnancy outcomes in women with Swyer-James-MacLeod syndrome, a rare obstructive lung disease. We present a case of a woman with this syndrome in pregnancy. Her baseline pulmonary function tests (PFT) demonstrated moderate airflow obstruction however she had excellent functional status and exercise tolerance. Her disease remained clinically stable in pregnancy. PFTs demonstrated slight worsening of her obstruction with forced expiratory volume in one second (FEV1). 59% and FEV1/FVC ratio 64%. She was diagnosed with gestational diabetes requiring metformin and insulin. Her labor and delivery was uncomplicated with vaginal delivery of a live male at term with no maternal respiratory complications. She did have a delayed postpartum hemorrhage requiring a D&C procedure. This case report demonstrates women with Swyer-James-MacLeod syndrome can have a successful pregnancy and need not avoid pregnancy if desired.

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