Background: People with Down syndrome seem to have a lower physical fitness than their peers without disabilities. Objective: The aim of this study was to examine the relationship of gross motor proficiency to body composition in children with Down syndrome through the Bruininks-Oseretsky Test of Motor Proficiency-Second Edition (BOT-2). Subjects and Methods: Twenty children with Down syndrome, aged from 6 to 9 years old of both sexes were participated in this study. They were selected from the public and special needs schools, Cairo government. Assessment of gross motor proficiency using Burininks-Osertsky Test of Motor Proficiency-Second Edition was performed. Results: The results of this study indicated that gross motor proficiency, after controlling for age and gender, is negatively associated with health-related measures including waist circumference, while there was weak non significance relationship between gross motor proficiency and Body Mass Index. Conclusion: From the obtained result of this study, it could be concluded that there is a significant correlation between gross motor proficiency and waist circumference in children with Down syndrome.