Aim: The World Health Organization’s International Classification of Functioning, Disability and Health adapted for children and young people (ICF-CY) is a framework for describing and classifying health and health-related states. The aim of the present study was to review literature on neurofibromatosis type 1 (NF1) using ICF-CY guidelines and to highlight findings about the quality of life of children with NF1. Method: Electronic databases were searched to identify studies involving children with NF1. Eligible studies were classified according to ICF-CY categories. Results: Children with NF1 have a variety of cognitive and other deficits. However, very little information is available on the impact of these deficits on their daily life. Interpretation: Despite the broad range of functional and structural deficits in children with NF1, the functional assessment of these children remains largely unexplored. Future studies should aim at evaluating the participation of children with NF1 in various situations, using tools with high real-world validity.