Urachal carcinoma is a rare cancer, accounting for only about 1% of bladder-related malignancies and 0.01% of all cancers in adults globally. Urachal adenocarcinoma is an aggressive cancer with poor prognosis. Treatment strategies are lacking due to the scarcity of cases and related studies. Differential diagnosis of urachal carcinoma can be difficult, and requires comprehensive imaging and pathological studies. Surgery is considered first-line treatment, while chemotherapy and/or radiation have limited impact on overall survival. The most common clinical symptoms are hematuria, mucinuria, and a palpable lower abdominal mass. To raise awareness of this rare cancer, we report urachal adenocarcinoma in a 49-year-old man who underwent imaging, pathological diagnosis, and surgical management.