Primary hyperparathyroidism (PHPT) is caused by a dysfunction intrinsic to the parathyroid gland’s ability to respond to serum calcium levels appropriately resulting in over-secretion of PTH and downstream multi-organ responses, which in turn increase serum calcium above the normal level. Children are more likely to present with symptoms of hypercalcemia including bone pain from resorption, urolithiasis/nephrolithiasis, and nonspecific signs such as fatigue and muscle weakness. In children, primary hyperparathyroidism is more often the presenting manifestation of a syndrome than in adults. Neonatal severe hyperparathyroidism is caused by homozygous mutations of the CaSR gene resulting in complete gland insensitivity to serum calcium levels. Early diagnosis has drastically improved survival in these patients. Primary hyperparathyroidism in the pediatric population is most commonly caused by a single adenoma. Multi-gland disease is associated with syndromes such as several of the multiple endocrine neoplasia syndromes. Ultrasonography, thallium-technetium dual-isotope scintigraphy, and Tc99m-sestamibi can successfully localize a majority of single adenomas. Accurate adenoma localization has resulted in the rise of directed and less-invasive parathyroid surgery as a favorable alternative over the traditional bilateral central neck exploration. Data comparing these approaches are sparse in the pediatric population. A robust data set in adults suggests that directed approaches may be effective and less morbid.