Abstract: Objective To compare the clinical manifestations between parathyroid hormone insensitivity syndrome (PHP) and hypoparathyroidism (HPP). Methods Thirty-six patients with PHP and 62 patients with HPP hospitalized in Chinese PLA General Hospital from January 1993 to December 2015 were included in this study. All clinical data were collected and analyzed. Results The age, height, vitamin D₃, thyroxine and free thyroxine, luteinizing hormone and follicle-stimulating hormone in PHP group were signigicantly lower than those in HPP group (P< 0.05); Alkaline phosphates(194.1±125.9) U/L vs (83.6±50.5) U/L and amino-terminal propeptide of total typeⅠcollagen(409.7±433.4)μg/L vs (51.5±109)μg/L, bone resorption markersbeta collagen degradation products(1.8±1.1) ng/ml vs (0.4±0.4) ng/ml and urinary calcium(1.1±1.1) mmol/24 h vs (2.0±1.8) mmol/24 h, as well as bone turnover markers-osteocalcin(68.4±56.2) ng/ml vs (13.7±15.3) ng/ml in PHP group were much higher than those in HPP group (P< 0.05, respectively). The incidence of tetany (80.6% vs 54.8%), abnormal stature (55.6% vs 8.1%) and ectopic calcification (69.4% vs 30.6%) in PHP group were higher than those in HPP group. Patients in both groups were administrated with calcium carbonate D₃(600 mg, 1-2/d) and calcitriol (0.25 μg, 1-2/d). The frequency of tetany decreased significantly after treatment. Conclusion Patients with PHP or HPP both present with hypoparathyroidism with different degree of severity. The diagnosis of PHP should be firstly considered in young patients with family history of such presentations and with obvious abnormal stature, thyroid function, gonad function, and abnormal bone biochemical markers.