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Solitary crossed renal ectopia (SCRE) represents an exceedingly rare congenital disorder. Although skeletal and genitourinary abnormalities most commonly accompany this condition, vesicoureteral reflux (VUR) has been described in only a few cases. Here, we present two unique cases of SCRE complicated by high-grade VUR concomitant with posterior urethral valve in one case and hypospadias in the other one. We also provide a brief review of the literature on this subject.