<h4>Purpose</h4>To describe a case of hereditary spastic ataxia (HSP) presenting with childhood optic nerve atrophy and report a novel homozygous variant in the <i>SPG7</i> gene.<h4>Observations</h4>A 57-year-old man suffering from progressive optic nerve atrophy since childhood eventually underwent genetic testing. The gene discussed is SPG7; the disease is hereditary spastic paraplegia.