Renal involvement is uncommon in MELAS patients, mostly presenting as either tubulopathy, interstitial nephritis, FSGS (most common), or cystic pathology [4-7]. It is most linked to MT-TL1 mutation (m.3243A>G) [8]. Kidney transplant in these patients has been associated with a 100% survival rate with a follow-up range extending up to 12 years [9]. However, MT-ND5 mutation-associated adult onset nephropathy is extremely rare and is described in only nine cases so far with variable manifestations consisting of a mix of FSGS and tubulointerstitial disease (Table 4) [10-14]. This evidence concerns the gene MT-ND5 and MELAS.