A rare presentation of unilateral periureteral renal lymphangiomatosis

Renal lymphangiomatosis is a rare developmental malformation of the perirenal lymphatic system. We report a unique case with unilateral massive periureteral involvement in addition to intrarenal and peripelvic lymphangiomatosis. Although this is a rare entity, it should be considered in patients with peripelvic or periureteric cystic lesions as it may affect appropriate management and follow-up. This case report reviews the imaging features of this entity and a comprehensive literature review and discussion about the entity will be provided.