The management of the difficult airway is one of the most challenging situations an anesthesiologist may encounter. The pediatric patient provides unique challenges such as lack of cooperation, rapid desaturation while apneic, and the presence of syndromes with craniofacial manifestations not frequently encountered in adults. These craniofacial manifestations may include mandibular hypoplasia, facial asymmetry, and limited mouth opening, to name a few. This chapter presents a case of a 5-year-old boy with Klippel-Feil syndrome and discusses the different aspects involved when dealing with a difficult airway in a pediatric patient, including the use of fiberoptic devices, neuromuscular blockade, and sedative premedication.