<h4>Objective</h4>This study aims to summarize the diagnostic and treatment experience of a case of Hypoparathyroidism-Sensorineural Hearing Loss-Renal Dysplasia (HDR) syndrome caused by a heterozygous mutation in the GATA3 gene.<h4>Methods</h4>The diagnostic and treatment process of the patient with HDR syndrome in our hospital was compared and analyzed.<h4>Results</h4>A 9-month-old male infant with a history of poor physical condition and increased susceptibility to infections. The gene discussed is GATA3; the disease is hypoparathyroidism.