BACKGROUND: Castleman's disease (CD) is a very rare disorder of unknown etiology that is characterized by masses of lymphoid tissue. METHODS: The records of all patients with a histological diagnosis of CD who were seen at the Hospital Nacional Cayetano Heredia and the Instituto Nacional de Enfermedades Neoplásicas between 1985 and 2003 and 1985 and 2001, respectively, were reviewed. RESULTS: The study included 10 patients. The age of onset ranged from 5 to 65 years. Nine patients met the criteria for localized CD; six of them were asymptomatic. The most common sites of presentation were the cervical lymph nodes (n=5), submaxilar lymph nodes (n=2), parotid gland (n=1), and lung (n=1). Eight patients had histological evidence of the hyaline-vascular variant and one had the plasma-cell variant. The primary treatment was complete surgical resection. One patient met the criteria for multicentric CD; he was asymptomatic, had histological evidence of the plasma-cell variant, and was treated with combination chemotherapy. All ten patients are currently alive with no evidence of recurrence. CONCLUSION: The localized form of CD presents as progressive, painless, slow-growing lymph node enlargement that is generally asymptomatic. The locations most commonly involved in the localized form are the cervical lymph nodes, followed by the submaxillary lymph nodes, where it poses a diagnostic challenge to the clinician because it tends to mimic other head and neck diseases. Localized CD is almost always of the hyaline-vascular variant and complete surgical excision of the tumor allows full recovery in all cases.