Background: In typical development, primarily contextual factors at the family level are clearly associated with child developmental outcomes. In contrast, knowledge about the contextual factors influencing the developmental characteristics of children (0-12 years) with severe to profound intellectual disability is scarce. Method: Through computerised searches of 4 bibliographic databases, 54 studies were identified. Results: The included studies were characterised by the use of small study populations and nonvalidated research instruments, as well as by strong research designs and detailed reporting of individual results. The majority of the studies evaluated procedures based on behaviourist learning theory, mostly aimed at reducing nonadaptive behaviour or stimulating choice behaviour. The studied factors proved to be generally effective. Conclusions: Family and parenting factors are one of the most striking gaps in the current literature about the development of children with intellectual disability. Also, further research on long-term developmental changes and bidirectional influences is necessary.