Vascular anomalies are rare lesions of diverse nature that may affect the head and neck region. Any mass in or around the upper airway has the potential to obstruct or compromise it. The absolute priority, before etiologic treatment, is the evaluation of the risk for the airway and its management. Prenatal diagnosis of an upper airway obstruction requires a planned delivery in a center having a specialized team experienced in managing a compromised feto-neonatal airway, and who could perform an ex-utero intrapartum treatment to secure the airway. Even after birth, the airway remains central in the patient's overall management. Signs and symptoms of airway compromise must be evaluated keeping in mind the specific requirements of infants and small children and being aware that rapid worsening may occur. The treatment is then tailored to the patient and his lesion with the goal of improving symptoms while avoiding treatment-related complications. Maintaining reasonable expectations by the patient and families are part of a successful management. Cure is achievable for small and localized lesions, but symptom relief and mitigation of functional, esthetic and psychological impairments is the goal for large and complex lesions. If a tracheotomy was required, decannulation is one of the primary management goals.