OBJECTIVES: We describe a case of hyaline vascular type Castleman’s disease with unifocal presentation as retroperitoneal mass. We describe the pathological and radiological findings and present a bibliographic review.METHODS: Castleman’s disease is a rare benign disease of unknown etiology characterized by a lymphoproliferative disorder. Two clinical types have been described: localized and multicentric. Isolated retroperitoneal involvement is uncommon.RESULTS: 39-year-old female who complained of back pain. Abdominal CT scan identified a 5 cm retroperitoneal interaortocaval mass. Complete laparoscopic excision was performed. Pathological study showed localized angiofollicular hyperplasia (hyaline vascular type Castleman’s disease).CONCLUSIONS: The presentation of Castleman’s disease as isolated retroperitonealmass is quite rare and should be distinguished from other retroperitoneal lesions of malignant character. The treatment of choice is surgery, providing a definitive and curative diagnosis. Operative biopsy may be useful.