We summarize important metabolic consequences and long-term complications associated with enterocystoplasty with particular emphasis on the pediatric patient with genitourinary abnormalities. A directed MEDLINE literature review for metabolic and long-term complications following enterocystoplasty was performed. Information gained through the published literature and from our database was reviewed and summarized to provide the reader with a thorough review of the subject. Bowel is not a perfect tissue for substitution or augmentation and its use to treat functionally and structurally compromised bladders is associated with several metabolic consequences and long-term complications. Metabolic acidosis is the most common metabolic abnormality seen. The rates and severity of these complications vary, although they may have a profound impact on patient quality of life after enterocystoplasty. The metabolic consequences and long-term complications associated with enterocystoplasty are important clinical features of this intervention. Careful consideration should be given to them prior to pursuing enterocystoplasty.