The first clinical description of 4 individuals with MEGDEL (3‐methylglutaconic aciduria, dystonia–deafness, encephalopathy, Leigh‐like) syndrome was published in 2006.1 In 2012, biallelic variants in SERAC1 (serine active site containing 1) were shown to cause this autosomal‐recessive deafness–dystonia disorder.2 Soon afterward, with the description of liver involvement as an additional clinical feature, hepatopathy was incorporated into the acronym (MEGDHEL; Mendelian Inheritance in Man [MIM] #614739).3 The gene discussed is SERAC1; the disease is deafness.