PHACE syndrome consists of the constellation of manifestations including Posterior fossa anomalies of the brain (most commonly Dandy-Walker malformations), Hemangiomas of the face and scalp, Arterial abnormalities, Cardiac defects, and Eye anomalies. We present the case of a patient who presented with respiratory distress at birth secondary to a large nasal glioma. She was subsequently found to have a ventricular septal defect (VSD), a facial hemangioma, and a malformation of the eye and optic nerve head. The nasal glioma, which extended to the cribriform plate, has not been described in this syndrome. The tumor was resected through a coronal incision, midline nasal bone osteotomy, and a retrograde dissection from the nasal bones to the anterior skull base. Glioma of the skull base is a novel and serious manifestation of this uncommon condition.