A congenital nasolacrimal duct cyst is an uncommon condition in the newborn usually treated by ophthalmologists. Prolapse or expansion of the cyst into the nose may lead to respiratory distress and difficulty in feeding as newborns are obligate nose breathers, which needs the involvement of the Otolaryngologist in diagnosis and management. The authors report a series of 8 children presenting with a congenital nasolacrimal duct cyst and dacryocoele and highlight the importance of endoscopic nasal examination of newborns presenting with respiratory problems. Diagnostic studies included intranasal endoscopy and CT scanning of the nasolacrimal system and nose. All the patients were treated by endoscopic marsupialization of the cyst. Nasolacrimal duct abnormality should be considered in the differential diagnosis of neonatal respiratory distress and nasal obstruction. Nasal endoscopy is essential in the work-up of all children with nasal obstruction and respiratory distress. CT scanning is the investigative method of choice. Endoscopic marsupialization followed by lacrimal duct irrigation is effective in the treatment of congenital nasolacrimal duct cyst and results in complete resolution of symptoms.