The authors describe the case of a 49 years old female with a progressive tetraparesis with two years of evolution, whose diagnostic study revealed a polymyositis/systemic sclerosis overlap syndrome. The patient was completely dependent and bedridden and had pulmonary, gastrointestinal, skin and bone involvement. This case intends to report and document that the use of “last-line” treatments such as intravenous immunoglobulins are effective and can be a therapy of great impact on the quality of life of the patient, even when apparently irreversible injury is established.