Etiology of neonatal seizures (NNS) is diverse and hypocalcemia is one of the treatable causes. Neonatal hypocalcemia (NHC) due to congenital hypoparathyroidism, either permanent or transient, is extremely rare. Its biochemical abnormalities include hypocalcemia, hyperphosphatemia and low levels of intact parathyroid hormone (PTH). Isolated congenital hypoparathyroidism in which deficiency of PTH has no association with maternal, syndromic or endocrine defects is a very rare entity. We are reporting a case of a newborn who presented with seizures on the 5th day of life and later on investigations revealed hypocalcaemia due to isolated congenital hypoparathyroidism.