The diagnosis of congenital cytomegalovirus (CMV) infection is best established by isolating the virus from urine or saliva within the first 2 weeks of life. Detection beyond this point may reflect perinatal acquisition of CMV. Cytomegalovirus is usually transmitted by direct human-to-human contact through vertical or horizontal routes. Infected persons can excrete CMV in urine, saliva, semen, cervical secretions, or breast milk. Because the virus establishes latent infections, blood products and solid organs can also transmit CMV. In the surgical literature, intestinal CMV-infected cells in infants have prevalently been associated with neonatal necrotizing enterocolitis, and only very few cases of primary CMV infection of the gastrointestinal tract of surgical interest in immunocompetent neonates have been reported. We describe a neonate with congenital or perinatal CMV infection with gastrointestinal involvement who developed a colonic stricture and manifested a clinical picture simulating Hirschsprung's disease. The intestinal lesion was a localized segmental CMV infection of the colon in which inflammation dominated the histopathologic finding. Chorioretinitis was also present.