Benign paroxysmal torticollis (BPT) is a rare paroxysmal dyskinesia and 1 of the childhood periodic syndromes presenting with recurrent stereotypic episodes of torticollis, usually accompanied with some of the nonheadache features of migraine such as vomiting and ataxia. Although the nature of BPT may seem benign, its recurrent episodes can mimic attacks of epilepsy and expose the infant to unnecessary hospitalization and adverse effects of inappropriate medications. There is no approved medication for the disease, but a few studies have suggested that cyproheptadine is useful. However, use of this agent has not been confirmed as effective for these patients, and the safe dosage for children aged <2 years has not yet been established. We report 4 patients who exhibited a successful response to treatment with topiramate (their episodes of BPT stopped). Considering the underlying relation of BPT with migraine, the satisfactory response of our patients to topiramate, and the safety of this medication in neonates and children, topiramate seems to be an effective and safe medication for the reduction and elimination of BPT episodes. In addition, 1 of our case subjects (patient 4) confirmed this finding by exhibiting an explicit dependence in the regularity and duration of her attacks with topiramate. Topiramate seems to be an effective medication for the prophylaxis of BPT episodes. Further studies and clinical trials are recommended.