This article reports findings from a literature review on literacy instruction for adolescents with significant cognitive disabilities. Nineteen empirical studies published between 1975 and 2011 were included in the review and analyzed based on characteristics of interventions. The results were examined to determine whether the state of research in this area indicates a comprehensive approach to literacy for adolescents with significant cognitive disabilities. Results indicate that vocabulary instruction through sight word acquisition is still prevalent, instruction is not comprehensive, and instruction does not address recommendations from the field for adolescent literacy. However, innovative approaches to literacy instruction for this population are beginning to be explored. The current state of research on reading practices for adolescents with significant cognitive disabilities, implications for practice, and suggestions for new directions in research are discussed.