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Urinary tract anomalies are common and comprise about 20% to 30% of total congenital anomalies. This spectrum consists of many different anomalies of the urinary tract that may be syndromic or nonsyndromic with different etiologies. In this case report, a patient with single kidney and urinary tract signs is introduced that was diagnosed accidentally. The finding of different anomalies in different organ systems should lead us to examination of the intactness of the urinary tract. In these disorders, if there is no need for immediate intervention, long-term follow-up can be helpful to postpone chronic kidney disease progression. [ABSTRACT FROM AUTHOR]