Background: Neonatal severe hyperparathyroidism is an extremely rare condition that presents in the first few months of life. Recognition at the early stage followed by prompt surgical intervention are of vital importance for survival. Hypotonia, respiratory distress, lethargy and delay in growth and development occur in association with the elevated serum parathormone levels and hypercalcemia. Definitive therapy involves total parathyroidectomy.

Case Description: We are presenting a case with Neonatal severe hyperparathyroidism, who underwent total parathyroidectomy. The patient had been followed up with medical therapy until. Parathormone levels rapidly declined following total parathyroidectomy and the patient was discharged with full recovery.

Literature Review: Sestamibi scintigraphy might fail to show an ectopic parathyroid gland. In such conditions, on table confirmation of parathyroid glands by frozen section and Intraoperative parathormone monitoring is vital at this point. Persistently elevated parathormone levels might suggest a remnant parathyroid tissue at the surgical site or an ectopic parathyroid gland that should be excised.

Clinical Relavance: Neonatal severe hyperparathyroidism is a life-threatening disease. In the present case surgery performed at the early is life-saving due to the failure of medical therapy to control the disease.