One of the uncommon urogenital malformations in males, which presents late in the second or third decades of life, is the congenital malformation of the seminal vesicle. Zinner syndrome is a rare condition comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction, and ipsilateral ejaculatory duct obstruction. Very few cases in the literature describe the malignant change in seminal vesicle cyst in Zinner syndrome. To the best of our knowledge, this is the first report of a papillary neoplasm of the seminal vesicle cyst developed in a patient with Zinner syndrome.
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