The corpus callosum is the brain structure that divides the right and left hemispheres. It consists of approximately 200 million neural fibers and is responsible for interhemispheric transfer of information and higher-order cognition. Congenital agenesis of the corpus callosum (ACC) is a condition that involves partial or complete absence of the corpus callosum from birth (i.e., not acquired). A systematic review of the literature on cognitive and developmental features of children and adolescents with ACC from 1969 through 2011 was conducted, and two hundred seventy-three studies were selected for initial review. Specific studies are described in this paper, and the salient findings are discussed. The findings are then contextualized within the framework of the International Classification of Functioning for Children and Youth in order to summarize the research and illustrate the utility of that instrument. Implications for future research and clinical practice are discussed.