Hemiparkinson-hemiatrophy (HP-HA) syndrome is a rare form of secondary parkinsonism. We present a case of a 19-year-old, left-handed man with subcortical heterotopia. He had hemiatrophy and hemiparkinsonian symptoms on his right side. We described the clinical, neuroradiological and neurophysiologic findings including transcranial magnetic stimulation study. These findings were compatible with HP-HA syndrome and his symptoms were markedly improved with levodopa treatment.