Introduction Transitional cell carcinoma of the bladder (TCCB) is uncommon in the pediatric population, and its etiology, natural history and epigenetics remain poorly understood. We aim to describe six cases of TCCB in pediatric patients and discuss the state of the art in the management and follow-up of the patients with this uncommon early presentation. Methods The clinicopathological data of 6 patients with TCCB who underwent transurethral resection of bladder tumor (TURBT) were obtained

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... m our institutional database. The patient data were collected retrospectively. A review of the literature was performed, and the most relevant and trending data were analyzed. Results A total of 6 patients (4 female, 2 male) were treated at our institution between 2004 and 2019. The mean age of the sample was 12 years, and the presenting symptoms were macroscopic hematuria (3 cases), suprapubic pain (2 cases), and 1 case was an incidental finding during pelvic ultrasonography. The long-term follow-up (median follow-up of 61 months) did not reveal recurrence. Conclusion Transitional cell carcinoma of the bladder rarely presents in the pediatric population. Genetic and epigenetic anomalies have been proposed as causes, as well as carcinogenic exposure. The reported cases tend to have a good prognosis, and most are non-invasive at the diagnosis. Follow-up protocols are still lacking, as well as molecular insights on tumor development and prognostic markers.