Restrictive lung disease is uncommon in pregnancy. We reviewed 15 pregnancies in 12 women with restrictive disease due to kyphoscoliosis, neuromuscular disease, or parenchymal lung disease. Median FVC was 40% predicted, and six women (50%) had an FVC < 1.0 L. In the 14 pregnancies in which at least two spirometry readings were available, FVC increased in three pregnancies, decreased in three, and remained stable in eight, with maximal changes of 0.4 L. Three women required supplemental oxygen, and one woman with neuromuscular disease required noninvasive ventilation. Premature delivery occurred in nine pregnancies (60%), and 10 deliveries (67%) were by cesarean section. Neuraxial anesthesia was used in 10 of 15 deliveries but was limited in the others by difficult spinal anatomy. There was no maternal or neonatal mortality. Women with restrictive lung disease tolerate pregnancy reasonably well, but many have premature delivery. A multidisciplinary approach is essential, with monitoring of spirometry and oxygenation and planning for labor and delivery.