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Lymphangioma is a rare benign tumor caused by failure in the development of the lymphatic communicating system. The corresponding nomenclature is confusing. In recent years ''renal lymphangiectasia'' is the preferred name. Although this disease may occur in any site of the body, the neck (75%) and axillary area (20%) are the most common sites, and the kidney is occasionally involved. We report a case of lymphangioma communicating with the urinary system in a 61-year-old man diagnosed by CT scan treated with nephrectomy and histological confirmation.
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