A young man with a history of recurrent respiratory tract infections for the past 8 years presented with generalised anasarca. He was found to have advanced renal disease (stage 4) and a profound proteinuria of 82.9 g/day. Further workup revealed the presence of advanced bronchiectatic changes in the lungs. Rectal biopsy showed the characteristic pattern of amyloid deposition. A diagnosis of systemic amyloidosis secondary to bronchiectasis was made. To the best of our knowledge no other cases of bronchiectasis-associated renal amyloid disease with such marked proteinuria have been reported in the literature. Our patient had a relatively short duration between the onset of his symptoms related to underlying bronchiectasis and his clinical presentation of renal amyloidosis. He had an aggressive course of disease and unfortunately died at a very young age.