Vol. 8, Issue 1, Part A (2026)

Primary Alveolar Proteinosis (PAP) is a rare lung disorder caused by decreased clearance and accumulation of surfactant. It is classified into primary, secondary, and congenital forms. Congenital PAP, linked to mutations (e.g., in SP-B, SP-C, ABCA3), is a rare, potentially fatal condition in newborns, often presenting as respiratory failure. Diagnosis relies on lung biopsy and bronchoalveolar lavage (BAL). We report a case of male neonate, born at 38+3 weeks was admitted to the NICU for respiratory distress. CT imaging suggested PAP and bronchoalveolar lavage (BAL) confirmed the diagnosis by demonstrating lipid-laden macrophages. The infant showed clinical improvement following BAL, supportive care and was discharged off respiratory support with adequate feeding and weight gain. This case highlights that, early diagnosis and management can lead to favourable outcomes in congenital PAP, potentially avoiding the need for whole lung lavage or transplantation.