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Pulmonary interstitial glycogenosis (PIG) is known to be associated with a wide variety of congenital conditions, though the extent to which PIG contributes to clinical presentation and outcomes in infants remains controversial. We describe two cases of infants with congenital anomalies and respiratory distress at birth who were diagnosed with PIG with differing clinical courses and response to methylprednisolone therapy. These cases highlight the importance of improved recognition of PIG and uncertainties about which patients may benefit from treatment.