Introduction: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition that results in neonatal respiratory difficulty. The purpose of this systematic review was to compare surgical outcomes of drilling versus dilation techniques in the treatment of CNPAS. Methods: Pubmed, Embase, and Cochrane Clinical Trials databases were searched for terms “congenital nasal pyriform aperture stenosis” or “pyriform aperture stenosis” from 2010 to 2021. Twenty-five studies were included that evaluated pediatric patients treated surgically for CNPAS with available outcomes data including complications, revisions, and length of stay. Results: A total of 51 patients with CNPAS were pooled from included studies. The median age was 29 days, 56.9% were female, and 54.9% were born full-term. The median pyriform aperture width before surgery was 5.00 mm (IQR = 4.10, 6.45). Forty (78.4%) patients underwent sublabial drilling, while 6 had a dilation procedure performed with hegar cervical dilators, 2 had a balloon dilation, and 3 were dilated with either an acrylic device, endotracheal tube, or bougie. There were no post-operative complications for 76.5% of patients, while a second surgery was required in 9 (17.6%) patients. The median length of stay was 11 days (IQR = 4, 26). No statistically significant difference was observed between sublabial drilling and surgical dilation techniques with respect to complications, need for revision surgery, or length of stay. Conclusion: Current literature is insufficient to determine if drilling or dilation is more effective in the treatment of CNPAS.