ABSTRACT
Castleman disease progresses with the enlargement of the affected lymph nodes and represented by a wide range of symptoms. This lymphoproliferative disease rarely affects the retroperitoneum. A patient with Klinefelter syndrome was admitted to our clinic following palpitation and sweat attacks. It was observed that the urinary catecholamine metabolites were elevated and a pararenal mass was found on the left side. The patient was directed to surgery with the paraganglioma pre-diagnosis after further examination. Histopathological examination of the excised mass confirmed the diagnosis of Castleman disease. The urinary catecholamine metabolites returned to near-normal levels at eight weeks after the surgery. We present a case of Castleman disease in a patient with Klinefelter syndrome, imitating paraganglioma as per the clinical, radiological and laboratory findings.