Paraneoplastic syndrome is defined as a group of symptoms accompanying malignant diseases that are not caused by the local presence of cancer cells in the affected organ or tissue. Although paraneoplastic syndrome is by definition induced by malignancy, there are single reports of paraneoplastic syndromes induced by benign tumors.1,2 We present a case of a 71-year-old male patient with systemic vasculitis, showing recurrent symptoms despite pharmacological treatment. Clinical and partial immunological improvement were achieved after the removal of thoracic schwannoma.