Chyluria is the passage of chyle in the urine. The cause seems to be the rupture of retroperitoneal lymphatics into the pyelocaliceal system, giving urine a milky appearance. This communication is caused by the obstruction of lymphatic drainage proximal to intestinal lacteals, resulting in dilatation of distal lymphatics and the eventual rupture of lymphatic vessels into the urinary collecting system.This condition, if left untreated, leads to significant morbidity because of hematochyluria, recurrent renal colic, nutritional problems due to protein losses and immunosuppression resulting from lymphocyturia.In this review, we summarize the state of the art of this condition and the newest treatments available.