Primary ciliary dyskinesia(PCD)is a hereditary disorder by mutations of genes related to the cilia. Over 70% of PCD patients suffer from neonatal respiratory symptoms. However, in cases without situs inversus or any family history, early diagnosis can be challenging. The present case is a girl born at 41 weeks weighing 3,400 g who was transferred to our NICU because of respiratory distress which developed on day 2 after birth. Chest radiograph showed an atelectasis of the right upper lobe, and heterotaxy syndrome was excluded by echography. On day 5, new atelectasis developed in the right middle and left upper lobes, which disappeared after physiotherapy. The patient needed oxygen administration until day 20, and had persistent nasal congestion and wet cough after discharge. PCD was suspected and confirmed by electron microscopy in nasal epithelium biopsy and genetic analysis at 3 months of age. There are many causes of neonatal respiratory distress. Among them, PCD should be included especially in cases with onset of several hours after birth, prolonged oxygen therapy and atelectasis unrelated to endotracheal intubation. Subsequent follow-up is recommended, and further specific investigations of PCD should be considered if there is year-round nasal congestion and wet cough.