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ImportanceMaternal hyperparathyroidism can be associated with significant maternal and fetal morbidity and fetal mortality. Because the maternal symptoms are typically nonspecific, the disorder may not be recognized leading to adverse pregnancy outcomes.ObjectiveThe aim of this study was to review the literature on the etiology/prevalence, pathophysiology, diagnosis, management (medical and surgical), and the maternal/neonatal complications associated with pregnancies complicated by hyperparathyroidism.Evidence AcquisitionA literature search was undertaken by our university librarian using the search engines PubMed and Web of Science. Search terms used included “hyperparathyroidism” AND “pregnancy” OR “pregnancy complications” OR “maternal.” The number of years searched was not limited, but the abstracts had to be in English.ResultsThere were 309 abstracts identified, 164 of which are the basis of this review. This includes 137 articles of the 269 individual case reports in the literature since the first case report in 1947. The articles and case reports reviewed the etiology, risk factors, diagnosis, management, complications, and maternal/fetal outcomes of pregnancies complicated by hyperparathyroidism.Conclusions and RelevanceUndiagnosed maternal hyperparathyroidism can result in critical maternal and fetal outcomes during pregnancy. This review highlights what is currently known about hyperparathyroidism during pregnancy to increase the awareness of this serious pregnancy disorder.Target AudienceObstetricians and gynecologists, and family physiciansLearning ObjectivesAfter completing this activity, the learner should be better able to identify the causes of hyperparathyroidism in pregnancy; explain its pathophysiology; detect the nonspecific symptoms/conditions; evaluate the medical and surgical management; and describe the maternal and fetal complications.