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Proximal renal tubular acidosis (P-RTA) occurs in mulple inherited metabolic condions; one of them is Mitochondrial disorder. Pearson syndrome, a type of mitochondrial cytopathy, presents with mull system involvement in the form of progressive external ophthalmoplegia, proximal myopathy, seizures, ataxia, stroke,renal tubular dysfuncon, hepaac, endocrine failure and sideroblassc anemia. Clinical features are variable due to heteroplasmy. L;iterature search shows that fewer than hundred casesfatcat:uodxbcuhyrhfteaehqtbsnv6ym