Objective: Barakat syndrome, also known as HDR syndrome, is a rare disorder caused by pathogenic variants in GATA3 characterized by hypoparathyroidism, sensorineural deafness, and renal anomalies. This case highlights a previously unreported GATA3 variant in a pediatric patient and underscores the diagnostic value of repeat genetic testing when clinical suspicion remains high. Case Presentation: An 11-year-old African American male presented with seizures due to severe hypocalcemia from hypoparathyroidism. Initial genetic testing was non-diagnostic. The patient was treated with calcium and calcitriol and discharged with outpatient endocrine follow-up. After a two-year lapse in care, he re-presented with recurrent hypocalcemia. Repeat testing using an expanded next-generation sequencing panel identified a novel pathogenic deletion encompassing exon 2 of the GATA3 gene, consistent with Barakat syndrome. Subsequent workup revealed mild bilateral sensorineural hearing loss and a simple renal cyst but normal renal function. The patient was re-established with endocrinology, nephrology, and audiology for long-term multidisciplinary management. Conclusion: This case adds to the expanding genotypic spectrum of GATA3-related HDR syndrome and illustrates the importance of updated genetic testing in cases with a suggestive clinical phenotype but initially negative results. Timely diagnosis facilitated targeted subspecialty care and avoided unnecessary investigations, emphasizing the need for ongoing evaluation in evolving genetic landscapes.