Dropped head syndrome (DHS) is an uncommon clinical syndrome, which requires complex diagnostic evaluation. A variety of neuromuscular and neurodegenerative disease can produce weakness of head extensor muscles and consequently lead to head drop. Inflammatory myopathy has been described as a cause of DHS, however head drop has only exceptionally been reported as being the presenting symptom of this disorder. We describe an original case of DHS as an initial and predominant manifestation of inflammatory myopathy with histopathological features of polymyositis, with an excellent response to immunosuppressive treatment.