Chorea is a rare manifestation of systemic lupus erythematosus (SLE). We report on a young patient with chorea who was diagnosed initially with rheumatic fever. Follow up and further evaluation confirmed the diagnosis of SLE and anti-phospholipid syndrome. Of special interest were the negative antiphospholipid (aPL) antibodies and the initial diagnosis of rheumatic fever which is still not uncommon problem in our region. The rarity of such presentation with joint and non specific increase of antistreptolysin O (ASO) titer might be the factors that led to an incorrect diagnosis. Early diagnosis and treatment of SLE and anti-phospholipid syndrome are very crucial and should be considered with such presentation.