Bilateral focal hand dystonia is the pathognomonic sign of Partington syndrome, fre- quently accompanied by intellectual disability, mouth and feet dystonia. However, few studies have focused on the treatment of this focal dystonia, making patient man- agement meticulous. Through a systematic review of the literature, we found that only levodopa and baclofen showed some symptomatic improvement, while pro- pranolol, gabapentin, and haloperidol were reported as ineffective. The descriptions in those studies were however imprecise and the amount of improvement rather mild, preventing definitive conclusions about their effectiveness. We present two cases of Partington syndrome featuring ARX gene mutations, hand dystonia, and other clinical signs. Various drug treatments were attempted, including levodopa, tri- hexyphenidyl, tetrabenazine, and benzodiazepines. Additionally, a blinded dystonia protocol was used to evaluate the efficacy of levodopa in one of the two patients. These cases provide an initial step towards better understanding and managing dystonia in Partington syndrome.