Congenital bony nasal cavity stenosis is caused by alterations of the normal embryological development of the nasal cavity. Depending on the site of the obstruction, the most important types of stenosis are: choanal atresia and stenosis, congenital nasal pyriform aperture stenosis, congenital midnasal stenosis, arhinia and nasal septum deviation. Although they are uncommon, they could be potentially life-threatening conditions that require early diagnosis and proper treatment. In case of neonatal nasal obstruction, appropriate differential diagnosis with other causes, such as rhinitis and sinonasal masses, are performed by nasal endoscopy and radiological exams. Treatment strategy consisting of medical nasal therapies and endoscopic or open nasal surgery should be tailored according to the types and the degree of the stenosis. When indicated, endoscopic endonasal approach is considered the most effective technique in neonates warranting minimal surgical invasiveness and maximum effect. In order to promote the management of these rare yet clinically relevant neonatal nasal breath disorders, we review the current trends in diagnosis and treatment of congenital bony nasal cavity stenosis.