Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass

The commonest skull manifestations in neurofibromatosis involve the orbit, with very few reports about occipital defects. We report a rare case of a 54-year-old lady with a massive plexiform neurofibroma extending from the auricular region down her left neck and into her shoulder, with an associated large left occipital and left petrous bone defect and extensive cerebellar meningo-encephalocoele, which presented with a relatively asymptomatic cervical mass and was treated with resection of the neurofibroma and advancement and rotational skin flaps.