We recommend performing complete and repeated screening for extrarenal manifestations in patients with biallelic pathogenic variants in COQ2, COQ6, and PDSS2 or presenting phenotype suggestive of CoQ10-related glomerulopathy (hearing deficit, encephalopathy, seizures, ataxia, hypotonia, motor/intelectual disability, elevated lactate levels, and diabetes). The gene discussed is PDSS2; the disease is lipoprotein glomerulopathy.