Neuroendocrine (NE) tumors of the bladder are infrequent. Among them, large cell NE tumor has been outlined only in case reports and short case series, which describes them as aggressive malignancies with dismal prognosis requiring a multidisciplinary approach. The case of an 82-year-old patient with muscle-invasive large cell NE carcinoma of the bladder associated with urothelial carcinoma in situ is presented. Despite combined treatment modality, relapse with metastatic disease was detected 12.5 months after the initial diagnosis. Thereafter, we review several published cases, and results of the collected data are compared with the largest studies published on this topic.