Agenesis of the corpus callosum is a common indication for fetal neurology consultation, increasingly identified through advances in fetal sonography and fetal magnetic resonance imaging. Despite improvements in diagnostic accuracy, prognostic counseling is challenging due to highly variable neurodevelopmental outcomes. Several factors contribute to neurodevelopmental outcome variability, including associated anomalies and etiologic considerations such as genetic, acquired, and environmental factors. This narrative review discusses existing literature on prenatal findings, postnatal outcomes, and comorbidities to provide practical guidelines for prenatal diagnosis, counseling, and postnatal management. Additionally, practice and research gaps are identified to advocate for guidelines to improve counseling, management, and optimization of outcomes for affected children and families.