Alhough mutations in SDHB, SDHC, and SDHD were associated with inherited PCC/PGL/HNPGL and mutations in SDHA were associated with autosomal recessive metabolic encephalopathy syndrome (Leigh syndrome) about 15 years ago (Horvath et al. 2006), the association of pathogenic germline SDHA mutations with inherited PCC was described only 5 years ago (Burnichon et al. Here, SDHA is linked to Leigh syndrome.