Introduction: This study investigated the functional ability of children with arthrogryposis, aged 6 months to 7.5 years, in South Africa, with the focus on the execution of age-appropriate self-care tasks. The level of independence, amount of caregiver assistance and modifications used during each task were ascertained, to assist in guiding occupational therapy intervention. Method: This study was based on a quantitative cross-sectional design, with 19 participants divided into age groups assessed using the Pediatric Evaluation of Disability Inventory (PEDI). Results: The infant and toddler group performed above expected levels in the self-care domain, whereas the preschool and school-aged group performed below the expected level. A significant correlation between the amount of caregiver assistance needed and the participants' level of independence was found especially in the preschool and school-aged group. There was little use of modifications even with occupational therapy intervention and participants relied on compensatory methods and assistance to execute self-care tasks. Conclusion: This study indicated that children with arthrogryposis need intervention to promote the independent execution of selfcare tasks, and reduce the need for caregiver assistance. Therapists are encouraged to make use of modifications in furthering the age-appropriate independence of this population.