Advances in knowledge and medical science have resulted in an increased life span and quality of life of patients with cystic fibrosis (CF). The median age of survival for CF patients is 32.3 years of age and patients 18 years of age or older now constitute one third of the total patients with CF. Because of these advances, a new patient population has emerged: the adolescents and young adults with CF. Adolescence is normally a time of great cognitive, social and developmental changes. Adolescents with CF not only have to deal with the normal changes expected, but also have to deal with the transition of assuming responsibility for their care from the parents and transitioning their care from a pediatric to an adult care team. Moreover, many of these young adults have to deal with the impact of the progressive deterioration of their CF disease. This review discusses issues of significance to this emerging patient population, including medical care, issues of disability, and psychosocial and other medical conditions associated with an increased life expectancy.