We describe the case of a 12-hour-old, full-term newborn girl referred to the Ear, Nose and Throat emergency team with increased work of breathing and stridor present at birth. Flexible nasendoscopy revealed a cystic laryngeal lesion obstructing the glottis that prompted securing of the airway with intubation and transfer to a tertiary paediatric centre. On further investigation with MRI and direct visualisation, the lesion was identified as a mixed macro/microcystic laryngeal lymphovascular malformation. The patient successfully underwent a series of microlaryngo–bronchoscopy and coblations of the laryngeal lesion with the aim of avoiding a tracheostomy. We describe the presentation, diagnosis and management of this rare condition in a paediatric case, along with a literature review of the subject.