Introduction: Sarcomatoid carcinoma of the urinary bladder is a rare bidirectional malignant neoplasm with epithelial and mesenchymal differentiation. The epithelial component is mainly high-grade urothelial carcinoma, and the mesenchymal component includes rhabdomyosarcoma. However, proper differential diagnosis of adult rhabdomyosarcomatous tumors of the bladder can be a challenge. Moreover, low-grade urothelial carcinoma as the epithelial component of sarcomatoid carcinoma has not been reported. Case presentation: A 64-year-old Asian man with a history of transurethral resection of low-grade urothelial carcinoma of the bladder visited our department with complaints of frequent urination and macroscopic hematuria. Computed tomography and magnetic resonance imaging demonstrated a large mass located in the anterior wall of the bladder. Pathological diagnosis of transurethral biopsy was low-grade, non-invasive papillary urothelial carcinoma, and tumor tissue was removed by total cystectomy. Immunohistochemical studies and fluorescence in situ hybridization assay of the resected neoplastic tissue revealed extensive rhabdomyosarcomatous differentiation causing the formation of a large pedunculated polyp with a papillary appearance of recurrent low-grade urothelial carcinoma. No evidence of recurrence was detected during 2 years of follow-up without further treatment. Conclusions: Urothelial carcinoma of the urinary bladder with extensive rhabdomyosarcomatous differentiation is rare, but it should be considered in the differential diagnosis even when urothelial carcinoma coexisting with a rhabdomyosarcomatous component is low-grade and non-invasive.