This systematic review evaluates the validity, reliability, and clinical utility of outcome measures used to assess self-care skills among children with congenital musculoskeletal conditions and assesses the applicability of these measures for children with osteochondrodysplasia aged 0-12 years. Electronic databases were searched to identify self-care assessments that addressed the self-care domain as defined by of the International Classification of Function Disability and included children with osteochondrodysplasia. Ten measures were identified and three met the inclusion criteria: the Functional Independence Measure for Children (WeeFIM), the Activities Scale for Kids (ASK), and the Pediatric Evaluation of Disability Inventory (PEDI). Although psychometric data specific to children with osteochondrodysplasia are limited, adequate to excellent reliability and evidence of validity were reported for all three instruments for children with physical disabilities. Further evaluation of psychometric properties of self-care instruments specifically for children with osteochondrodysplasia would be beneficial to help identify instruments that will assist with improved assessment and management.