The pediatric primary care provider in the medical home has a central and unique role in the care of children with spina bifida. The primary care provider addresses not only the typical issues of preventive and acute health care but also the needs specific to these children. Optimal care requires communication and comanagement with pediatric medical and developmental subspecialists, surgical specialists, therapists, and community providers. The medical home provider is essential in supporting the family and advocating for the child from the time of entry into the practice through adolescence, which includes transition and transfer to adult health care. This report reviews aspects of care specific to the infant with spina bifida (particularly myelomeningocele) that will facilitate optimal medical, functional, and developmental outcomes.