Abstract :Background: A noval radiologic sign in patients with renal failure and UE with metabolic acidosis has recently been identified as the lentiform fork sign. On magnetic resonance imaging (MRI), the ”lentiform fork sign” has been described as bilateral symmetrical hyperintensities in the basal ganglia encircled by a hyperintese rim delineating the lentiform nucleus. Changes in uremic solute retention, aberrant blood-brain barrier transport, disorderd vascular reactivity, altered electrolyte and acid-base balance, and altered hormone metabolism are the most likely causes of the condition.Case presentation : 56-year-old man with end-stage renal disease was brought to the emergency room for a progressive change in mental status and involuntary arm movements over the previous five days, which were also accompanied by mild dyspnea.A brain MRI was performed, and it revealed hyperintensity on T2/FLAIR in the white matter surrounding the basal ganglia. The haloperidol was stopped, and there more dialysis sessions were carried out.Conclusion : intensified hemodialysis and glycemic control are the cornerstones of treating DUS with likely reversible clinical symptoms and remission of imaging abnormalities.