RESUMEN
Chyluria despite pseudo- banality may pose several diagnostic and therapeutical problems. Its etiopathogenesis is unclear, too. Prolonged loss of the lymph observed in patients with chyluria due to inherited anomalies of lymphatic system may lead to serious deficits and homeostasis disorders. Basing on these thesis, the authors present two patients with chyluria due to inherited anomaly of extraperitoneal lymphatic system and review available literature. Particular role in the clinical follow-up of these patients plays their immunologic status. The authors did note deficiency of cell-mediated immunity measured with skin tests and lymphocyte T blastic transformation test, and increasing IgG deficit, and particularly annoying IgA deficit in the patient with long-lasting, massive chyluria.