Summary

Congenital anomalies of the kidney and urinary tract (CAKUTs) occur in 3–6 per 1000 live births, accounting for most cases of paediatric end-stage kidney disease.¹ However, the molecular basis of CAKUT and anomalies of the external genitalia is poorly understood. We, herein, describe a case with left recurrent epididymo-orchitis with a coexistent urethral duplication and an ectopic ureter with an ipsilateral non-functioning kidney, which is, to the best of our knowledge, the first reported case of its kind. This case may bring about a paradigm shift in our comprehension of the development of the two entities. Understanding the pathogenesis may help develop preventive and renal preservation strategies. The Sonic hedgehog gene and bone morphogenetic protein 4 play crucial roles in preventing anomalies of the ureters and the external genitalia. In this article, we look at possible molecular pathways that could explain the synchronicity of this rare entity.