It is caused by genetic variants in <i>FA2H</i>, which encodes fatty acid 2-hydroxylase.<h4>Objective</h4>To report the clinical, electrophysiological, radiological, and genetic profile of patients diagnosed with FAHN.<h4>Methods</h4>We performed a retrospective chart review of genetically proven cases of FAHN from our database.<h4>Results</h4>We identified eight patients (6 females) with genetically proven FAHN. Here, FA2H is linked to fatty acid hydroxylase-associated neurodegeneration.