Here, we report a lethal homoallelic truncating variant in ARL6IP1 manifesting with classical features of HSP and additionally with dysmorphic features, developmental delay, microcephaly, neuropathy, leukoencephalopathy, partial agenesis of corpus callosum, seizures, high grade gastroesophageal reflux disease, and respiratory distress in two affected members of a consanguineous Saudi family. Here, ARL6IP1 is linked to hereditary spastic paraplegia.