COQ9 mutations are responsible for renal tubulopathy with extra‐renal manifestations.5 Some cases of isolated SRNS have been described in patients with COQ2 and COQ8B mutations.3, 7, 9, 10, 11 The link between CoQ10 and renal disease was first established in 2000 by Rotig et al when three siblings were diagnosed with a complex clinical syndrome characterized by progressive encephalomyopathy and SRNS. Here, COQ2 is linked to kidney disorder.