We describe a case of distal renal tubular acidosis in a 20-year-old woman with a history of glue sniffing. On admission she complained of nausea and somnolence. Severe hypokalemia and hyperchloremic metabolic acidosis with normal anion gap were noted. Her urinary osmolar gap and anion gap results were consistent with presence of renal tubular acidosis. Bicarbonate loading test along with urine PCO2 confirmed that she had type 1 renal tubular acidosis due to hydrogen ion secretion defect. Immunohistochemical staining of renal biopsy specimen with anti H+-ATPase antibody showed absence of proton pump in the collecting duct. Thus a case of distal renal tubular acidosis in association with glue sniffing is reported with a review of literatures.