While the great majority of the cases of primary hyperparathyroidism present with consistent and persistent hypercalcemia in laboratory test profile, a relatively new and rarer phenotype characterized by normal serum calcium concentrations in the face of persistently above-normal parathyroid hormone (PTH) levels has been recognized in a small proportion of patients in the past two decades and given the term normocalcemic hyperparathyroidism. Mostly, patients having this rare disorder could only be diagnosed incidentally or serendipitously during assessment of bone mineral disorders (osteoporosis or fragility fracture) or urolithiasis when serum calcium and PTH levels are measured for etiology finding. Long-term follow-up studies of this clinical entity in these patients have observed the natural course to be either stationary with persistent normocalcemia or developing into classical phenotype of hyperparathyroidism with time with steady or fluctuating pattern toward definitive hypercalcemia, and hence regarded by some investigators as a mild form of the classical phenotype of primary hyperparathyroidism at its initiation. Despite the status of normocalcemia, exposure to the excessive PTH concentrations of multiple tissues and organs could incur target organ damages in the long run which may manifest with disorders of bone mass or quality, fracture, or nephrolithiasis that in turn leads to impairment in renal function. Due to the largely unpredictable natural course and the odds of abovementioned complications as may well-known to occur in classic primary hyperparathyroidism, it is prudent to keep periodic and continual follow-up and, when indicated, timely treatment of this specific phenotype of hyperparathyroidism. Surgical intervention by removal of identifiable parathyroid gland lesion(s) (hyperplasia or adenoma), or when surgical treatment is not feasible, pharmacological therapies aiming to inhibit bone resorption by osteoclasts with bisphosphonates to improve bone mineral density and reduce risk of fracture, or to reduce PTH back to normal levels by calcimimitics through its effect on calcium-sensing receptors at the parathyroid gland in hope of reducing urolithiasis burden, are all options available to ameliorate the risk of target organ damage by the excessive concentrations of PTH.