OBJECTIVE: To report a case of xantho-granulomatous cystitis, a rare disease especially in the western countries (12 of the 16 cases reported were Japanese and Korean). METHODS: A case of xanthogranulomatous cystitis in a 34-year-old female is presented. Patient evaluation included histological, histochemical and immunohistochemical studies. RESULTS/CONCLUSIONS: The lesion was localized to the bladder dome, anatomically related with the urachus. It consisted of collections of histiocytes with lymphoplasmocytic infiltrate. The adhesions observed in the omentum in this case indicate a possible relationship with a previous inflammatory process. Surgery is advocated in most of the cases with good results.