Constipation in children is very common. Childhood constipation responds successfully to medical therapies, behavioral modifications, or a combination of both in approximately 70% of children within 2 years of diagnosis. Persistence of constipation is associated with fecal incontinence, recurrent fecal impactions, and significant emotional problems. Refractory constipation that interferes with both the child's function and the family's life requires extensive evaluations in order to uncover its cause and devise appropriate therapeutic strategies. Children with persistent constipation can be divided into three broad categories: 1) those who have functional constipation, 2) those affected by enteric neuromuscular disorders, and 3) those whose constipation is associated with neurologic handicaps. Treatment options vary widely. The majority of patients require more aggressive medical and more intense behavioral interventions. Others benefit from novel approaches, including surgical intervention.