Cytomegalovirus (CMV) is the most common congenitally acquired viral infection in the United States and is associated with significant morbidity and mortality. Primary CMV enterocolitis is well documented in immunocompromised patients, but remains rare in congenitally acquired infections. There are no universally accepted recommendations for the treatment of CMV enterocolitis in the pediatric population. Case reports show varied dosing and length of treatment of either intravenously administered ganciclovir, orally administered valganciclovir, or a combination of both. We present a congenitally infected infant with primary CMV enterocolitis who was successfully treated with orally administered valganciclovir.