Cognitive problems are common in children with neurofibromatosis type 1 and they can often complicate treatment. The current literature review examines cognitive functioning in neurofibromatosis type 1, with a specific focus on executive functioning. This includes exploration of how deficits in executive functioning are expressed in children with neurofibromatosis type 1 and how these deficits contrast with ADHD. The value of investigating subcomponents of executive functioning is discussed, as are implications for effective treatment and future research. (Contains 3 tables.)