A homozygous frameshift variant in the PODXL gene (podocalyxin-like, MIM*602632) has been described in three siblings from an Indian consanguineous family, who developed a pure levodopa-responsive Parkinsonism manifested at 13–17 years of age, later complicated by dyskinesia and off-dystonia, with no additional signs. Here, PODXL is linked to Dystonia.