In this report, we present a patient with BD, amyloidosis and end-stage-renal-disease and discuss the literature on BD and amyloidosis. Forty-four patients with BD and amyloidosis are described in the literature. The type of amyloid fibrils were studied in 27 of this 44 patients and all were AA. The interval between the onset of first symptom of BD and the diagnosis of amyloidosis ranged from one to 27 years. This clinical observation and AA type amyloid fibrils in BD suggest that amyloidosis is secondary to inflammation. Behçet's disease should be considered in the differential diagnosis of AA amyloidosis.