Background: Congenital tracheobiliary or bronchobiliary fistulae are rare developmental malformations associated with high morbidity and mortality primarily because of concomitant anomalies as well as delays in diagnosis. Technical Report: We report a case of a neonate who presented with bilious emesis and progressive respiratory failure requiring bronchoscopy and fluoroscopic bronchography for diagnosis with successful management through video-assisted thoracoscopic surgery. We also review the published literature on tracheobiliary fistula. Conclusion: Use of video-assisted thoracoscopic surgery for fistula resection has been reported in only one other neonate and provides a clinically less invasive option compared with open surgical ligation and resection. To our knowledge, this is the first English language report of thoracoscopic resection in a neonate with congenital tracheobiliary fistula.