Neurofibromatosis 1 is one of the more common inheritable disorders that surgeons may encounter. A plethora of systemic associations, both benign and malignant, can affect these patients, and an acute awareness of these associations is essential for proper surgical care. A complete review of this disorder from the surgical perspective follows, highlighting the importance of this awareness. A brief review on the management and follow-up of surgical malignancies associated with this disorder is included.