Background: The basaloid carcinoma has not been previously described in the urinary bladder. Case presentation: A 61-year-old man presented with hematuria. The patient had a history of skin tumor with 37 basal cell carcinomas, which had often been treated with surgical excision. The cystoscopy showed a superficial 6 mm papillary lesion. A transurethral resection (TUR) instillation was performed, and the histological analysis revealed an invasive basaloid carcinoma pT1. Because of this uncommon histological aspect, a second Look TUR was conducted and showed no tumor and was followed by an intravesical Bacillus Calmette-Guerin therapy. Knowing the patient presented two major aspects of the Gorlin Syndrome, a genetic analysis of patched mutation had been done but showed no mutation of the gene. After 5 years of cystoscopy follow-up, the patient presented no recurrence of the tumor in the bladder. Conclusion: The basaloid carcinoma is an exceptional variant tumor in the urinary bladder.

Keywords: Basaloid carcinoma, urinary bladder, PTCH gene, urinary bladder neoplasm, histological variant