Children born with myelomeningocele (MMC), the most severe form of spina bifida, face multiple challenges throughout their life span. Neurogenic bowel, neurogenic bladder, Arnold-Chiari II malformation, and hydrocephalus are common complications that have implications for elimination, feeding, and learning. Nutrition education and intervention that use the concepts of family-centered care beginning in infancy and continuing throughout childhood, adolescence, and adulthood are important. These can help maximize wellness, fitness, and independence in children with MMC, as constipation, dysphagia, and obesity are common comorbidities seen in individuals with MMC. This article reviews the literature on nutrition assessment and intervention in this unique population to assist nutrition professionals in caring for children with MMC.