Infantile myofibromatosis is an uncommon and benign condition presenting in the neonatal period. It is self-limiting disease that may present as a localised or generalised process. Various examples of this entity have been reported in the literature. This report describes a neonate with a rapidly growing oropharyngeal lesion obstructing the airway that had the typical histological features of an infantile myofibroma. This case report highlights that a solitary myofibroma may be incredibly extensive making complete excision impossible and can be particularly challenging to manage in terms of airway stabilisation.