Urachal adenocarcinoma is a rare cancer and is treated based on the experience from case series and expert opinion as no randomized studies have been performed. This report adds to the current literature the experience of a patient with locally advanced urachal adenocarcinoma who was treated with combination of neoadjuvant gemcitabine/ cisplatin chemotherapy, surgery and adjuvant chemotherapy and has obtained a long recurrence free survival currently for more than 5 years. Although 5-FU-based chemotherapy is favored by many experts in the treatment of metastatic urachal adenocarcinoma, gemcitabine-based regimens have produced partial responses in metastatic disease and have been used in peri-operative treatment with a manageable adverse effect profile. A brief discussion of molecular lesions in urachal carcinomas and of the emerging role of targeted therapies is included in the current report.