Objectives　To analyze the clinical characteristics, diagnosis and treatment of systemic lupus erythematosus (SLE) with chorea as the main manifestation. Methods　The clinical manifestations, laboratory examinations and treatment of one pediatric patient with main presentation as chorea diagnosed with SLE in the year of 2014 were studied. The related literatures were reviewed. Result　A 14 years old female, was admitted with the main complaint of choreatic movements. The physical examination and laboratory tests indicated multi-system involvements (anemia, proteinuria, skin and mucous membrane damage and chorea). Combined with history of hemolytic anemia, positive antinuclear, anti-doublestranded (ds) DNA and anti-cardiolipin (aCL) antibodies, and multiple lesions in magnetic resonance imaging (MRI) scan, the diagnosis of SLE and NPSLE was confirmed. After the treatment of gamma globulin, methylprednisolone, and Aripiprazole, the symptoms were relieved. Conclusion　 SLE should be considered as a possible cause of chorea.