Abstract Objective The corpus callosum is a major white matter pathway of the brain that coordinates the transfer of information between both cerebral hemispheres. Children with complete agenesis of the corpus callosum (ACC) are at increased risk for neurodevelopmental disorders, epilepsy, and genetic abnormalities. Method This case study presents the neuropsychological profile of a 6-year-old girl in the 1st grade who was born with complete ACC and presented with a history of attention problems and behavioral-emotional difficulties. Results Neuropsychological testing revealed mild to severe deficits in attention, executive functioning, and self-regulation in the context of average intellectual functioning and broadly average to above-average academic achievement. Conclusions School recommendations included establishing a 504 plan and weekly counseling sessions with the school social worker in order to provide accommodations to support the child’s attention difficulties in the academic setting. Recommendations for genetic testing and ongoing monitoring by the patient’s neurologist were suggested given the higher rates of genetic abnormalities and seizures in children with ACC as these conditions can adversely impact neurodevelopmental outcomes. Medication management, as well as private behavior therapy with a parent-training component, were recommended for interventions to address the child’s deficits with attention and behavioral regulation. This case study demonstrates the need for prompt neuropsychological evaluation for children with ACC in order to more efficiently facilitate access to targeted assessments and treatments that can lead to improved outcomes.