Primary epithelial tumors of the bladder are extremely rare in children and adolescents. We report a case of transitional cell carcinoma in an adolescent with Turner's syndrome. The patient presented with intermittent gross hematuria and mild dysuria. A mass was identified on the trigone of the bladder near the left ureteral orifice, and histological evaluation revealed grade II to III in situ papillary transitional cell carcinoma with foci of submucosal invasion. Transurethral resection of the tumor was performed, and the patient remains free of disease 2 1/2 years later. The literature on transitional cell carcinoma of the bladder in children is reviewed.