This study aimed to examine the levels, demographic differences, and associations between body image (BI) and quality of life (QOL) among Japanese adolescents with congenital physical disabilities. A self-report questionnaire was administered to 107 Japanese adolescents with congenital physical disabilities, which included demographic variables, the Multidimensional Body-Self Relations Questionnaire, and the Japanese version of the World Health Organization Quality of Life Brief Version. Data were analyzed using Cronbach’s α coefficient, descriptive statistics, t-tests, one-way ANOVA, and Pearson’s correlation coefficient. The mean BI score was 2.96 ± 0.39. The domain-specific mean scores were as follows: health evaluation (3.49 ± 0.67), health orientation (3.11 ± 0.57), fitness evaluation (3.03 ± 0.93), fitness orientation (3.02 ± 0.70), appearance orientation (2.70 ± 0.61), and appearance evaluation (2.61 ± 0.61). The mean QOL score was 3.51 ± 0.50. The domain-specific mean scores were as follows: social relationships (3.61 ± 0.91), environment (3.60 ± 0.59), physical (3.46 ± 0.55), and psychological (3.42 ± 0.63). BI significantly varied by gender, school level, type of disability, and activities of daily living (ADLs), while QOL varied by school level and ADLs. All BI domains, except appearance orientation (investments for enhancing appearance), were positively correlated with QOL. Our findings suggest that school level and ADLs are key predictors of both BI and QOL among Japanese adolescents with congenital physical disabilities and that physical-fitness- and health-related BI are closely associated with QOL.