Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with lymphoproliferative tumors, and sometimes with a very rare tumor, Castleman's disease (CD). PNP can present as a variety of dermatological diseases, and so far, only limited studies of PNP caused by CD have been reported, resulting in its higher possibility of misdiagnosis. Because of the variability of clinical presentation of PNP caused by CD, selection of the appropriate therapeutic approach remains unclear. To investigate the efficacy of surgery to patients with PNP caused by localized CD, the clinical, laboratory and pathological data of 5 patients with PNP caused by localized CD, 3 females and 2 males, aged 34 years (ranging from 29 to 42), with the tumor size from 6 x 4 x 4 cm(3) to 8 x 8 x 7 cm(3), located in the mediastinum (n = 3) and retroperitoneum (n = 2), were compared before and after surgery. All patients underwent surgery, and the diagnosis was confirmed by pathological examination. Surgery significantly improved mucosal lesions, cured skin lesions and decreased serum pemphigus autoantibody 6 months after surgery. Most patients were followed up for 6-48 months, and they all had no clinical or radiological recurrence and remain disease free. Therefore, surgery is an effective approach to cure patients with PNP caused by localized CD.