Pulsating orbital plexiform neurofibroma and optic nerve glioma

A 13-year old Nigerian boy presented with a painless swelling which had grown, over ten years, into a pulsatile monstrous deformity of the left side of the face. Histology of the excised tissue showed a plexiform neurofibroma. It manifested with cutaneous, ocular and bony features. There was also a left eye optic nerve glioma. The case is presented and used to highlight the clinicopathological manifestations of orbital plexiform neurofibroma.