The clinical phenotypes of primary CoQ10-deficient patients are broader than initially reported in 1989 [33], including (i) a multisystem disorder with steroid-resistant nephrotic syndrome as the main clinical manifestation (COQ1-PDSS2) [34], (COQ2) [35], (COQ6) [36] and (ADCK4) [37]; (ii) a multisystem disorder without nephrotic syndrome (COQ1-PDSS1) [38], (COQ9) [39] and (COQ7) [40]; (iii) cerebellar ataxia (COQ8-ADCK3) [41,42,43,44,45,46,47]; and (iv) myopathy and encephalopathy (COQ4) [48,49,50]. The gene discussed is COQ8A; the disease is nephrotic syndrome.