Background: Protein-rich element binding protein A (PURA) syndrome is a genetic neurodevelopmental disorder that results in profound neurodevelopmental delay and intellectual disability. Few cases of PURA syndrome have been recognized in neonatal life, partly due to heterogenous clinical presentation, low clinical suspicion, and symptom progression that results in a more thorough workup later in life. Case Presentation: We report the case of a newborn who presented with hypotonia, feeding difficulties, and respiratory distress within the first hours of birth. Neonatal screening, infectious workup, and imaging were unremarkable. Clinical exome sequencing revealed de-novo, a pathogenic variant in the PURA gene. The patient received comprehensive rehabilitative care and social support. At one-year follow-up, the patient continued to experience feeding difficulties and apneic events and demonstrated significant neurodevelopmental delay. Conclusion: This report documents the consideration and diagnosis of PURA syndrome in the neonatal period and describes the implications of early diagnosis on prompt symptomatic management and patient outcomes at 1-year follow-up. [ABSTRACT FROM AUTHOR]