We present a case of Zinner syndrome (ZS), where we were fortunate to diagnose a child with this rare syndrome immediately after birth. Gross hydronephrosis was observed during the prenatal period on ultrasound, and further imaging after birth confirmed the presence of a multicystic dysplastic kidney and seminal vesicle cyst. The majority of cases of ZS is asymptomatic; however, symptoms relating to urination, ejaculation or infertility may present later on in life and so regular follow-up is required to ensure interventions can be carried out if such symptoms do occur.
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