From the extensive investigations of Dr. Fuller Albright has emerged our present-day understanding of the pathologic physiology of the clinical syndromes related to the functional states of the parathyroid glands (1). The clinical and biochemical manifestations of hyper- and hypoparathyroidism have been well documented in the medical literature, and the perplexities regarding these two conditions have for the most part been resolved. The nomenclature in connection with hypoparathyroidism has, however, become more complicated and confusing following the introduction of such neologic terms as pseudohypoparathyroidism in 1942 (2) and pseudo-pseudohypoparathyroidism in 1952 (3) by Albright and his co-workers. The classical picture of hypoparathyroidism is that of a clinical state of tetany due to disturbances of calcium and phosphorus metabolism. The biochemical changes may be depicted as follows: (a) increased renal tubular re-absorption of phosphorus; (b) decreased urinary excretion of phosphorus; (c) elevation of serum phosphorus concentration; (d) alteration of solubility ratio of calcium and phosphate ions; (e) fall of serum calcium concentration; (f) diminished resorption of bone; (g) supersaturation of body fluids with calcium-phosphate and soft-tissue calcifications. These changes are reversed following the administration of parathyroid extract. The deficiency of parathyroid hormone may be incident to thyroidectomy or idiopathic (4, 14). The association with moniliasis has been reported, although a causal relationship has not been established (15, 16). In 1942, Albright, Burnett, Smith, and Parson (2) described a group of 3 patients with clinical and biochemical alterations of hypoparathyroidism who did not respond to exogenous parathyroid extract and whose parathyroid tissue showed a normal histologic picture (or hyperplasia) on biopsy. In addition, these patients exhibited a number of developmental anomalies, such as round facies, short stature, brachydactyly, ectopic calcifications, and ectodermal abnormalities. Because of the unresponsiveness to parathyroid extract, the syndrome was termed pseudohypoparathyroidism in contradistinction to hypoparathyroidism, in which this response is exaggerated. Ten years later, Albright, Forbes, and Henneman (3) reported a case with physical defects similar to those of pseudohypothyroidism but no evidence of parathyroid dysfunction, and with normal response to parathyroid extract. For such patients the term pseudo-pseudohypoparathyroidism was coined. Since the original publications, a number of papers have appeared (5, 7–14, 17, 18), which make it clear that the physical stigmata of pseudo- and pseudo-pseudohypoparathyroidism (including brachymetacarpals and metatarsals, ectopic calcifications, round facies, short thick-set body habitus, and lenticular opacity) vary from patient to patient.