Newborn airway related diseases are one of the biggest challenges that an anesthesiologist can face during his medical practice.This case report describes a complete airway obstruction in a neonate with a laryngeal sarcoma who underwent various diagnostic procedures, finally receiving a total laryngectomy as part of his oncological treatment.We document the perioperative care during the laryngectomy, a procedure that has not yet been reported in literature to the date.We share our experience on how to confront such challenge and review the current literature on laryngeal cancer in the pediatric population.