Giant plexiform neurofibroma and suboccipital meningocele manifesting as segmental neurofibromatosis

A 34-year-old woman presented with segmental neurofibromatosis manifesting as a soft lump with a large café-au-lait macule on her occipital region and neck. Magnetic resonance imaging showed a thick skin tumor in the occipital region and posterior neck, and a suboccipital meningocele which seemed to have no association with her symptoms. Biopsy lead to a histological diagnosis of giant plexiform neurofibroma. During biopsy, massive local bleeding occurred and hemostasis was achieved by electrocautery and meticulous suture ligation. The postoperative course was uneventful and observation was continued for both the giant plexiform neurofibroma and the meningocele.