The observation of comorbid testicular tumor in males with exstrophy-epispadias complex should prompt a preventive health examination after puberty, which gives these patients the opportunity for further appropriate diagnostics and treatment if necessary. Biopsy is recommended for sonographically detected intratesticular lesions. Organ sparing procedures are worth considering, especially when stromal tumors with favorable outcome are discovered. However, current oncologic principles must be strictly followed. Although the etiology and true incidence of testicular tumors in exstrophy-epispadias complex are still unclear, our findings highlight the importance of long-term followup in patients with exstrophy-epispadias complex.