We report a case of idiopathic inflammatory myopathy accompanied with the presence of anti-Jo1 antibodies and complicated by diffuse parenchymal lung disease (antisynthetase syndrome). Efficacy of different therapeutic agents, including corticosteroids, cyclophosphamide and cyclosporin A, is described. We present beneficial effect of cyclosporin A and corticosteroids regimen, and confirm no benefits of plasmapheresis. An interesting association of the disease onset with toxoplasmosis infection is discussed and difficulties regarding diagnosis of cardiac involvement are addressed. We also review other case reports of this rare disorder of poor prognosis.