Our purpose in this systematic review was to identify, evaluate, and summarize the single-case research design studies examining behavioral interventions to address self-injurious behavior for young children with autism spectrum disorder. We identified 24 studies published between 2000 and 2016 that met the inclusion criteria. We reviewed and coded each study across five content areas using systematic procedures. These content areas consisted of the methodological quality of single-case research designs, children's demographic information, conditions under which the intervention was implemented, characteristics of intervention, and outcomes. Our findings indicate that procedural integrity data were reported for only one study and social validity for two studies. The majority of studies were implemented by researchers or therapists in the school setting. For 24 of 30 children included in the studies reviewed, the authors taught a replacement behavior selected on the basis of an assessment conducted prior to intervention. All authors reported a decrease in the self-injurious behavior post-intervention. A limited number of authors examined generalization and maintenance of low rates of self-injurious behavior. Limitations of the present review and future directions and implications for research and practice are discussed.