Objective To investigate the difference and treatment strategy of malignancy-associated dermatomyositis and other para-neoplastic neurological syndromes (PNS).Methods The clinical characteristics of a patient with malignancy-associated dermatomyositis and poly radiculoneuropathy was reported and the relevant literature was reviewed.Results Patients with dermatomyositis had increased risk of malignancies,and should routinely screened.Dermatomyositis and polyradiculoneuropathy was clinically similar,but could rarely be seen in the same malignant patient.Malignancy -associated dermatomyositis and PNS had similar pathogenesis.The treatment strategy of both was similar.Malignancy specific treatment should be initiated and immune suppressive agents should be prescribed concurrently.Conclusion Rheumatolgists should aware the association between dermatomyositis and potential underlying malignancies.Multiple para-neoplastic syndromes could be seen in the same patient,but the diagnosis should be considered as one.