OBJECTIVE To study the clinical manifestations of gastrointestinal cytomegalovirus disease in children with human immunodeficiency virus infection. METHODS Review of clinical records of eight human immunodeficiency virus-infected children and histopathologically confirmed gastrointestinal cytomegalovirus disease from 1995 to 2001. RESULTS Six of the eight children were younger than 1 year. The most common clinical presentations were fever and chronic diarrhea. Lower gastrointestinal hemorrhage and bowel perforation were noted in four and three patients, respectively. The colon was the most commonly affected site, followed by the small bowel and esophagus. The diagnosis was established by histopathology, obtained during endoscopy and surgery. Mucosal edema, erythema, and ulcer comprised the most common endoscopic findings. Two patients with fever, chronic diarrhea, and lower gastrointestinal bleeding developed remission after being treated with a 14-day course of ganciclovir. CONCLUSIONS Gastrointestinal cytomegalovirus disease can result in serious life-threatening complications, such as bowel perforation and massive gastrointestinal bleeding. Patients with chronic diarrhea and fever of unidentified cause might benefit from gastrointestinal endoscopy for early diagnosis and treatment. Although ganciclovir does not eradicate the infection and relapses are frequent, this treatment can prevent complications and reduce morbidity.