Levetiracetam (LEV) efficacy in the treatment of chorea in Huntington disease, paroxysmal nonkinesigenic dyskinesia, paroxysmal kinesigenic choreoathetosis, and dyskinetic cerebral palsy was reported in some studies. We described a case of a child with Sydenham chorea treated with LEV. A 7.5-year-old male patient presented with chorea, orofacial dyskinesia, speech impairment, and irritability. Echocardiographic examination revealed mitral insufficiency. Sydenham chorea was diagnosed after excluding other diseases causing chorea. Although his choreiform movements were decreased substantially with haloperidol treatment, speech impairment, orofacial dyskinesia, and light chorea were continued. Therefore, on day 9, LEV was added, and his complaints resolved in a few days. The severity of the chorea according to the Universidade Federal de Minas Gerais Sydenham's Chorea Rating Scale decreased from 47 to 5 points after LEV treatment. Thus, on day 13, the dose of haloperidol was reduced and gradually discontinued within 4 days. Symptoms did not reoccur. The follow-up at 1.5 months revealed recurrence of complaints due to discontinuation of LEV by parents. Signs and symptoms were regressed completely within 1 week after LEV retreatment. We suggest that LEV with fewer adverse effects comparing to other drugs may be considered to be a good alternative in the treatment of Sydenham chorea.