Heliox therapy, alone or combined with noninvasive ventilation, has successfully been used in the treatment of infants and children with respiratory disease such as airway obstruction. We describe the case of a 1-month-old infant with severe upper respiratory airway obstruction (approximately 80 %) secondary to multiple cervical hemangiomatosis effectively treated with either Heliox alone or combined with nasal continuous positive airway pressure. This treatment provided symptomatic relief and facilitated diagnosis and subsequent application of specific treatment; the patient was free of signs of respiratory distress and the use of aggressive treatments such as endotracheal intubation or tracheal cannulation was avoided. Heliox can constitute a multipurpose, safe and useful tool in the noninvasive management of infants and children with serious obstructive respiratory disease.