We report the effectiveness of a combination chemotherapy consisting of ifosfamide, 5-fluorouracil, etoposide and cisplatin (IFEP chemotherapy) against metastatic adenocarcinoma of the urachus. From April 1995 to November 2004, four patients with adenocarcinoma of the urachus who developed metastases after definitive surgery were treated with the IFEP chemotherapy followed by consolidative radiotherapy or salvage surgery in good responders. During the median follow-up period of 26 months, three of the four patients responded to the regimen (75%). In one patient with pulmonary metastases, four cycles of the IFEP chemotherapy, followed by stereotactic consolidative radiotherapy resulted in a complete remission for more than 8 years. Two patients achieved partial remission after the IFEP chemotherapy. We have demonstrated the IFEP chemotherapy is one of the most effective chemotherapy regimens against metastatic adenocarcinoma of the urachus. A multimodal treatment strategy, even with curative intent, can be considered when a sufficient effect was obtained by the IFEP chemotherapy.