Objective: To describe the clinical presentation, preoperative diagnostic possibilities, and treatment of cystic nephroma.
Methods: We describe a case of cystic nephroma in an adult male and show that, both in our patient and in the literature, a definitive diagnosis can only be obtained postoperatively, even when there is a reasonable clinical suspicion.
Conclusions: Cystic nephroma is rare, and some authors consider it a questionable entity. However, it should be distinguished from renal cystic neoplasms, which can resemble the condition.