Behçet's Disease is one of the systemic vasculitis which has an unknown etiology. Secondary Amiloidosis is a rare complication of Behçet's syndrome. In this article, we discussed a patient, who was accepted in the hospital as a nephrotic syndrome after detailed investigations diagnosed as a Behçet's syndrome. In that patient, we determined secondary amiloidosis by kidney biopsy. Being a very rare association, we also looked over the literature.

In that article, by the help of this case report we will review the last considerations about Amc. It is concluded that, Amc is not a disease but a terminology defining multipl congenital contractures, and is accepted that Amc occurs during fetal development because of mobilisation, limitation of the joints (the limitation of joint mobilisation). This is a rare picture, and generally characterised by stiffness of joints, flexion contractures of distal proximal joints and columna vertebralis, hipoplasia and/or aplasia of muscles and fibrous tissue.