Reversible acute symmetrical basal ganglial lesion on magnetic resonance imaging and/or computed tomography in cases of diabetic nephropathy and chronic renal failure exhibiting acute onset of movement abnormalities like chorea is a very rare entity. It has characteristic clinical and imaging features. Only 29 cases are described in the literature, including the current one. These cases are predominantly Asian patients from the Far East and only one Asian Indian patient has been described. We report the second Asian Indian case of this condition and describe its various clinical and imaging features. Our aim is to educate the clinicians and radiologists about this condition, so that more such cases can be detected.