Abstract Aims: To describe the clinical course of infants recovering spontaneously from diaphragmatic paralysis due to perinatal phrenic nerve injury as well as those that underwent plication of the diaphragm. Methods: Between 1990 and 2006, 14 newborns admitted to the Neonatal Intensive Care Unit (NICU) of the Wilhelmina Children's Hospital in Utrecht, The Netherlands, were diagnosed with diaphragmatic paralysis due to obstetric phrenic nerve injury. The clinical and follow-up data were studied retrospectively. Results: Four infants recovered spontaneously and could be weaned from mechanical ventilation within nine days without further treatment. Plication of the diaphragm was performed in 10 infants because of failure to wean from ventilatory support or serious persistent respiratory distress. Time between birth and plication ranged from 10 to 51 days, with a median of 19 days. Satisfactory respiratory outcome was achieved in 86% of the cases. Conclusions: The minority of infants suffering from diaphragmatic paralysis due to perinatal phrenic nerve injury recovers spontaneously. Infants who fail to wean from ventilatory support and undergo early plication have a quick recovery and can be extubated successfully within a few days.