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Lentiform fork sign is a rare neuroradiological abnormality with bilateral basal ganglion changes. It is seen in a variety of conditions characterized by metabolic acidosis, mostly seen with uremia of diabetic end-stage renal disease. Metabolic acidosis associated with uremia of acute pancreatitis has never been reported to be associated with this sign. Features of uremic encephalopathy are reversible with hemodialysis. The onset of worsening neurological symptoms along with a lack of improvement in encephalopathy despite hemodialysis must prompt the physician to do Magnetic Resonance Imaging (MRI) Brain in search of this sign. The scope of acute pancreatitis in its causation also needs to be investigated further. We report a case of acute kidney injury in a patient with acute pancreatitis who presented with metabolic acidosis and uremic encephalopathy. After initial improvement with hemodialysis, he gradually and sequentially went on to develop quadriplegia, generalized rigidity and distal upper limb tremor and later on full recovery along with lentiform fork sign on his MRI brain.