Background: There is a lack of evidence-based knowledge in paediatric occupational therapy about the effectiveness of interventions using daily activities as a treatment modality in improving children’s participation. Objective: This study aimed to evaluate the effectiveness of occupation-based and occupation-focused interventions in improving participation in everyday occupations for young children with a disability. Material and methods: A systematic review based on Joanna Briggs Institute methodology and critical appraisal tools was conducted. Six databases were searched for quantitative intervention studies aimed at improving participation in everyday occupations of young children with a disability through the use of everyday occupation. Results: The search yielded 3732 records, of which 13 studies met inclusion criteria. Ten studies met methodological quality criteria and were included in the synthesis, five randomised controlled trials and five quasi-experimental studies, involving a total of 424 children with a mean age of 6.5 years. The studies were classified into cognitive (n = 5), context-focussed (n = 2) and playgroup interventions (n = 3). Study quality ranged from low to moderate, only one study was rated high quality. Conclusions and significance: Occupation-based and occupation-focused interventions may have a positive effect on participation in everyday occupations for young children with a disability, but study design, risk of bias and insufficient reporting limit confidence in the body of evidence.