Primary systemic amyloidosis is an uncommon disease which presents with involvement of the kidney, heart, peripheral nervous system and liver. The involvement of skeletal muscles called amyloid myopathy is uncommon. We report a 74-year-old male who presented with progressively drooping shoulders followed by other muscular involvement without other organ involvement as a manifestation of amyloidosis. The patient was managed with melphalan, prednisolone and thalidomide with clinical improvement.