Sporadic primary hyperparathyroidism (PHPT) is primarily a disorder of post-menopausal women [1], [2], [3]. Hence, the incidence of PHPT among women of childbearing age is low. One large study from Israel estimated that out of 292024 women aged 20–40 years (reproductive age group) who underwent estimation of serum calcium, only 0.05 % had PHPT [4]. Accordingly, in the Western countries, PHPT in pregnancy accounts for less than 1 % of all PHPT cases [5], *[6], [7], [8], *[9]. On the contrary, PHPT in Asia presents early, often in the 4th or 5th decades of life [10]. Accordingly, a study by the authors reported that in a cohort of 386 Indian women with PHPT, 8 patients were pregnant equating to a prevalence of 2.1 % [11].

Primary hyperparathyroidism in pregnancy, often referred to as gestational PHPT, presents a unique challenge to the physician. The clinical manifestations of gestational PHPT are diverse, often non-specific, and vary in severity, making early diagnosis difficult [12]. Besides, the physiological changes that occur during pregnancy, primarily those involving calcium metabolism, make biochemical diagnosis all the more complex. In addition, limitations regarding the use of radiological imaging during pregnancy make it challenging to localize the culprit parathyroid lesion in pregnancy.

Despite the challenges, timely treatment of PHPT in pregnancy is imperative. If left untreated, gestational PHPT may lead to complications in both the mother and the foetus. Maternal complications have been reported in 67 % of patients with gestational PHPT including a high miscarriage rate *[6], [13]. Foetal and neonatal complications, reported to be as high as 80 %, include intrauterine growth restriction (IUGR), prematurity, low birth weight (LBW), hypocalcemic tetany, and seizures [14]. It should however be noted that some recently conducted large-scale studies have shown no association between gestational PHPT and feto-maternal complications [4], [15].

The management of PHPT in pregnancy is also fraught with challenges, primarily because of the potential risks of medical and/or surgical management during pregnancy to the foetus. Furthermore, management decisions are complicated by the lack of evidence and currently, there are no randomized controlled trials pertaining to the treatment of PHPT in pregnancy. There exists no clarity on the best timing for medical or surgical intervention in pregnancy *[12], [16].

The present review summarizes the intricacies of the diagnosis and management of primary hyperparathyroidism in pregnancy.