Urachal cancer is very rare, accounting for only 0.5–2% of bladder-associated malignancies and 0.01% of all cancers in adults. It has an insidious appearance, an aggressive behavior and a poor prognosis. The most common symptoms are hematuria and the presence of a palpable hypogastric mass. The scarcity of cases and the low number of studies carried out explains the lack of an evidence-based management strategy, but it seems that surgical treatment (open, laparoscopy or robot-assisted) represents the gold standard, while neoadjuvant and adjuvant chemotherapy or radiotherapy has a limited impact on overall survival. Since mucinous cystadenocarcinoma of urachal origin is a very uncommon pathological condition the differential diagnosis may be difficult and pathological investigations have to elucidate this disorder. It is worth mentioning the psychological impact on the patient in addition to the medical aspects. A rare condition is associated with heightened risk for mental health and psychosocial difficulties and this must be taken into account in the subsequent follow-up of the patient. In order to increase awareness of this rare entity we report a case of a 40-year-old male with a urachal adenocarcinoma who was treated surgically, with a favorable outcome. We also perform a brief literature review about this type of tumor.