Hereditary renal amyloidosis is an autosomal dominant condition with 
considerable overlap with other amyloidosis types. Differential diagnosis is 
complicated, but is relevant for prognosis and treatment. We describe a patient 
with nephrotic syndrome and progressive renal failure, who had a mother with 
renal amiloidosis. Renal biopsy revealed amyloid deposits in glomerular space, 
with absence of light chains and protein AA. We suspected amyloidosis with 
fibrinogen A alpha chain deposits, which is the most frequent cause of 
hereditary amyloidosis in Europe, with a glomerular preferential affectation. 
However, the genetic study showed a novel mutation in apolipoprotein AI. On 
reviewing the biopsy of the patient's mother similar glomerular deposits were 
found, but there were significant deposits in the renal medulla as well, which 
is typical in APO AI amyloidosis. The diagnosis was confirmed by 
immunohistochemistry. Apo AI amyloidosis is characterized by slowly progressive 
renal disease and end-stage renal disease occurs aproximately 3 to 15 years from 
initial diagnosis. Renal transplantation offers an acceptable graft survival and 
in these patients with hepatorenal involvement simultaneous liver and kidney 
transplantation could be considered.