Tracheo-esophageal fistula (TEF) is a well known and a relatively common congenital anomaly (1 in 3,000 4,500 live births). Half of the patients with TEF may have other congenital anomalies, VATER syndrome. However, the presence of subglottic laryngeal stenosis in patients with TEF is uncommon. We report a case of TEF (type C) combined with subglottic laryngeal stenosis. We anesthetized a 2.74 kg neonate for reconstruction surgery. But, it was impossible to pass an uncuffed endotracheal tube (2.5 mm inner diameter and 3.6 mm outer diameter) beyond the vocal cord. A gastrostomy was performed only under mask ventilation, and emergent tracheostomy was done. Endotracheal endoscopy revealed TEF combined with subglottic laryngeal stenosis. In this case, she can be managed successfully by the performance of an early tracheostomy.