Background: Bladder ES/PNET is a rare entity with a few cases described in the literature. This article will focus on an atypical vesical presentation of this tumor. Summary: We report a case of Ewing's sarcoma arising in the bladder in a 37-year-old patient with type 2 diabetes who presented with clotting hematuria and irritative symptoms of the lower urinary tract. On clinical examination, a hard and mobile hypogastric mass was palpated. CT scan revealed an abdominopelvic mass with predominantly extravesical development. Cystoscopy showed apparent displacement of the vesical dome without clear lesions within it. Given this perplexing presentation, we performed mass excision with partial cystectomy. Anatomopathological, immunohistochemical, and molecular biology studies supported the diagnosis of Ewing's sarcoma of vesical origin. Unfortunately, the patient passed away one month later due to complications related to a digestive obstruction. Conclusion; The objective of our presentation was to describe an additional case that presented both diagnostic and therapeutic challenges, in order to stimulate further consideration and derive clear recommendations.