Primary systemic amyloidosis is a rare disease caused by abnormal deposition of amyloid protein in different organ systems and mainly involves the kidneys, heart, peripheral nerves, and liver. The kidney is one of the most frequently affected organs in systemic amyloidosis which typically presents with nephrotic syndrome. Cutaneous involvement in amyloidosis can occur as a skin limited disorder or as a manifestation of systemic amyloidosis. Various cutaneous lesions can be seen in systemic amyloidosis, especially in form of petechial, purpura, and ecchymosis. Nail dystrophy and bolus skin lesions are the rare manifestations of systemic amyloidosis. In this case, we report an Iranian 64 years old man with bolus skin lesions and nail dystrophy associated with systemic amyloidosis.