Recently, mitochondrial tryptophanyl‐tRNA synthetase, encoded by WARS2, has been associated with the phenotypes of intellectual disability, leukoencephalopathy and seizures.8, 9 Here, we describe a patient with pathogenic WARS2 mutations presenting with infantile‐onset, Levodopa‐responsive Parkinsonism. This evidence concerns the gene WARS2 and Parkinsonism.