While cystic dysplasia of the testis is rare, the awareness of the benign and malformative nature of this lesion is important to consider among other cystic testicular and para testicular lesions, first neoplasm. Its sonographic appearance and the recognition of simultaneously occurring renal anomalies should allow for prompt recognition in children with scrotal swelling or testicular enlargement. We describe a 10-year-old boy with right testicular swelling who underwent orchiectomy for cystic dysplasia of the testis.