Metastatic urachal cancer is a rare disease and subsequently, does not have a defined systemic treatment. Although urachal cancer is most commonly adenocarcinoma and histologically similar to colon cancer, treatment selection is usually based upon location (the proximity of the urachus to the bladder) with bladder cancer regimens the most commonly prescribed. We report a case of metastatic urachal cancer where the immunohistochemical profile's similarities to colon cancer led to treatment with colon cancer specific chemotherapy. Our case is the first to report urachal cancer treated with and responding to modified FOLFOX6. In the age of targeted therapy, where molecular biology drives treatment selection, our case highlights that in rare tumors, when evidence is often lacking, a common sense approach can often prevail.