During recent decades, primary hyperparathyroidism (pHPT) has appeared as one of the more common endocrine disorders. Previously, the disease was the obvious cause of severe, symptomatic bone disease, recurrent renal stones, and sometimes devastating muscular weakness. The condition often progressed rapidly and ultimately ended in renal insufficiency. Today, pHPT is frequently recognized in patients with less obvious symptoms and markedly slower disease progression. However, if thoroughly examined, many of these patients will also present typical symptoms and complications of the disease. Surgery in pHPT has also developed as a highly efficient procedure with low failure rate and few complications. Further, successful operation is likely to decrease the risk of developing long-term disturbances of calcium metabolism and recently recognized cardiovascular complications of the disease. However, in a group of generally elderly patients with especially mild hypercalcemia and no obvious symptoms, disease progression may be slow, and it is possible that some of these patients can be followed safely without surgery. These patients also constitute a majority of cases detected in population surveys. Pathophysiological studies of pHPT have revealed more or less disturbed secretory regulation as a characteristic feature of pathological parathyroid glands, and this accounts principally for the patients' hypercalcemia. This abnormality has been related to decreased expression or capacity of parathyroid cell surface receptors executing a crucial calcium-sensing function. Recent progress has also led to the identification of causes of a growth regulatory disturbance in pathological parathyroid glands. Exploration of molecular mechanisms behind these abnormalities are likely to further unveil disease characteristics and help explain differences in clinical symptoms and disease progression among the patients with pHPT.