The authors describe their experience with surgical treatment of benign rare lymph proliferation - Castlemans disease (CD). It occurs in unicentric and multicentric forms. The very low incidence of the disease makes it very difficult to design larger prospective studies. Cases of two leading localizations of the unicentric form of CD - intrathoracic and retroperitoneal with special emphasis on the preoperative diagnosis and imaging options are described. This article underlines the curative potential of surgical treatment where a complete resection of the affected lymph node leads to eradication in almost 100% of the cases. The discussion is focused on the forms of CD - different localization, clinical symptoms and course of disease. It discusses the differential diagnosis, particularly difficult in the multicentric form, emphasizing the need to exclude malignant lymphoma. The etiopathogenesis of the disease is presented, mentioning its association with HIV (Human Immunodeficiency Virus) infection and HHV-8 (Human herpers virus 8) infection and the importance of overproduction of proinflammatory cytokines. The importance of surgical therapy for the unicentric form of CD is highlighted as compared to the multicentric form, where the surgeon´s task involves taking a biopsy - required for an accurate diagnosis.Key words: Castlemans disease - lymphoproliferation - lymphadenopathy - surgical treatment.