The predominant cause of hereditary renal amyloidosis is a mutation of the 
fibrinogen Aalpha chain (AFib), the most common being the E526V mutation. The 
evolution towards terminal renal insufficiency is constant and raises the 
question of renal transplantation and the risk of recurrence. We describe the 
case of a Portuguese woman with the E526V mutation without any renal or hepatic 
history in her family which developed a nephrotic syndrome at the age of 35, 
followed by stage 5 renal insufficiency. Because of the risk of recurrence of 
amyloidosis on its transplant, we carried out a combined transplantation 
liver-kidney despite the absence of clinical or biological hepatic 
abnormalities. Four years later, the result is excellent and there is no sign of 
the disease on the new organs. This successful experience as well as the five 
other published cases of combined liver-kidney transplantation in Aalpha 
fibrinogen chain amyloidosis, demonstrates the feasibility and efficacy of this 
treatment in AFib amyloidosis.