SERAC1 deficiency (SERAC1, biallelic variants, autosomal recessive inheritance, MIM #614739), first described in 2006,47 is a rare mitochondrial disorder (prevalence 0.09: 100 00048) which presents with 3MGA with sensorineural hearing loss, variable hepatopathy, encephalopathy and Leigh syndrome spectrum.49, 50. Here, SERAC1 is linked to Leigh syndrome.