Localized granulomatous lesions are a rare form of cytomegalovirus (CMV) infection. An eleven week-old Caucasion boy had a parotid xanthogranuloma associated with CMV infection of the same salivary gland. There was no evidence of disseminated CMV infection or other xanthogranulomas, and the patient is entirely well one year after excision of the lesion. The intimate association of CMV-infected cells with the tumor suggests an etiologic relationship. Previously reported cases of localized granulomas associated with CMV infection are reviewed.