A longitudinal study of self-concept and functional independence in 22 adolescents with progressive muscular dystrophy is reported. 30 adolescents with progressive muscular dystrophy completed Korean Self-Concept Scale, Modified Barthel Index, and a questionnaire examining demographic and medical factors. Functional independence was measured by Modified Barthel Index. Two years later, 22 of the 30 adolescents with progressive muscular dystrophy completed same Korean Self-Concept Scale and Modified Barthel Index. Adolescents with progressive muscular dystrophy were not changed on self -concept scores between test and retest. At retest adolescents with progressive muscular dystrophy scored significantly lower than at test on Modified Barthel Index. Compared to scale norms, subjects had significantly lower Total Self, Physical Self, Social Self, Self Satisfaction, Self Behavior scores. Age, years of education, and functional independence were significantly related self-concept. Functional independence was significantly related years of education.