Choroid Plexus tumors are rare. Surgery and biopsy is diagnostic, and radiotherapy has been used as the treatment of choice for choroid plexus carcinoma (CPC) and recurrent choroid plexus papilloma (CPP). We report the first case of CPP responding to combination chemotherapy consisting of cisplatin, bleomycin and vinblastine (CBV). This chemotherapy regimen should be considered for future trials in patients with choroid plexus tumors and recurrence after surgery and/or radiotherapy.