Key Clinical MessageNested variant of urothelial carcinoma (NV‐UC) is an extremely rare cancer with a nonspecific presentation. It is usually identified at a late stage, which makes the treatment challenging. Herein we report the case of a 52‐year‐old woman with an advanced NV‐UC treated by anterior exenteration after a poor response to neoadjuvant chemotherapy. One year after completion of adjuvant radiotherapy, the patient remains disease‐free.