Urothelial carcinoma of the bladder with choriocarcinomatous features is a rare presentation among genitourinary cancers. In this study, the case of a 42-year-old woman who presented with menstrual irregularity and positive urine and serum beta-human chorionic gonadotropin tests is presented. Pelvic ultrasound showed no intrauterine pregnancy. Laparoscopy and hysteroscopy with dilatation and curettage were negative for evidence of trophoblastic tissue. Computed tomography of the abdomen and pelvis showed an intravesical fundal mass, with no evidence of extravesical disease. Cystoscopy and transurethral resection of the bladder tumor diagnosed an invasive high-grade urothelial carcinoma with trophoblastic differentiation and multiple foci of choriocarcinomatous morphology. The patient received 3 cycles of neoadjuvant chemotherapy with methotrexate, vinblastine, adriamycin and cisplatin and then underwent partial cystectomy, which was negative for any residual tumor. This is the first reported case of a positive urine pregnancy test leading to the diagnosis of urothelial carcinoma.