Amyloid nephropathy is an unusual manifestation of hereditary gelsolin amyloidosis and may present with proteinuria and progressive renal failure. We report the first case of renal transplantation in a patient with hereditary gelsolin amyloidosis complicated by end-stage renal disease. The patient was a 44-year-old man from the Northwest of Iran who had undergone hemodialysis for 1 year. He finally received a living, unrelated renal transplant. During a 6-year posttransplant period, the patient maintained stable allograft function without proteinuria. No significant infectious or cardiac complications were noted. Although a definite conclusion cannot be reached with a single case, this report may indicate that renal transplantation can be successfully attempted in patients with hereditary gelsolin amyloidosis and amyloid nephropathy. Renal transplantation has been performed in various hereditary, primary, and secondary amyloidoses. A brief review of this topic is presented.