Plexiform neurofibromatosis is an uncommon variant of neurofibromatosis type 1 (NF1) (Von Recklinghausen’s disease). There is a greater prevalence of neurofibromatosis 1 in patients with other neoplasms, such as rhabdomyosarcomas, gastrointestinal stromal tumors (GISTs), pheochromocytomas, carcinoid tumors and ganglioneuromas. We report the anesthetic implications of a case of a 33 year old patient with plexiform neurofibromatosis and a history of pheochromocytoma which was operated on previously. He presented with painless swelling on upper eye-lead since childhood and had multiple caféau-lait spots and neurofibromas on the trunk. The surgery was done in two sessions first on the plexiform neurofibroma of the eye followed by the operation on the brain mass.