Background: Parathyroid disease is uncommon in pregnancy. During pregnancy, multiple changes occur in the calcium regulating hormones which may make the diagnosis of primary hyperparathyroidism more challenging. Close monitoring of serum calcium during pregnancy is necessary in order to optimize maternal and fetal outcomes. In this review, we will describe the diagnosis and management of primary hyperparathyroidism during pregnancy. Methods: We searched MEDLINE, CINAHL, EMBASE and Google scholar bases from 1 January 1990 to 31 December 2020. Case reports, case series, book chapters and clinical guidelines were included in this review. Conclusions: Medical management options for primary hyperparathyroidism during pregnancy are severely limited due to inadequate safety data with the various potential therapies available, and surgery is advised during the 2nd trimester of pregnancy in the presence of severe hypercalcemia (calcium adjusted for albumin greater than 3.0 mmol/L (12.0 mg/dL)). Hypercalcemia should be avoided during pregnancy in order to minimize maternal and fetal complications.