Objective: To report a case of late presentation of alveolar capillary dysplasia and misalignment of pulmonary veins.
Design: Descriptive case report.
Setting: Pediatric intensive care unit of a tertiary care children's hospital.
Patient: Seven-week-old female infant with severe hypoxemic respiratory failure.
Conclusion: Alveolar capillary dysplasia with misalignment of pulmonary veins is a cause of primary pulmonary hypertension in newborns that can rarely present past the newborn period. Early lung biopsy can be helpful in establishing the diagnosis and avoiding ineffective, futile, and expensive therapeutic interventions.