Primary lymphedema is a rare disease caused by a disorder of lymphatic system. Clinical presentation and age at onset are variable. AA amyloidosis is usually due to chronic inflammatory diseases, malignant tumors or less frequently chronic infectious diseases. The appearance of AA amyloidosis is rare in the course of inflammatory skin diseases, and chronic leg ulcers have been rarely reported to cause AA type amyloidosis. We report here two cases of AA amyloidosis that presented with renal failure and nephrotic syndrome in patients with primary lymphedema-induced chronic leg ulcers. We aimed to reveal that there is a relation between lymphedema, chronic leg ulcers, AA amyloidosis and chronic kidney disease.