Two neonates with pyriform sinus fistula were presented. The first case was a male with a large cystic mass of the left neck, who was intubated at birth because of respiratory distress. The plain radiograph of the neck revealed that the cyst contained air. Respiratory distress was further deteriorated due to bilateral chyrothorax after starting feeding. The second case was a female with left cervical cyst and hoarseness. These cysts, which were completely excised, had connecting fistulae epithe-lialized with both squamous and columnar cells. Recurrence has not been observed for one year after surgery in each case. In three neonatal cases previously reported, two patients underwent temporary drainage and later excision. However, our experience suggested that primary excision can be safely performed at the neonatal period, which would make patients free from the desease.