Sjogren’s syndrome is a female dominated autoimmune disorder with diverse phenotypical expression. For most patients, disease runs an indolent course with sicca symptoms, musculoarticular pain and disabling fatigue. Distal real tubular acidosis is an extra-glandular manifestation of Sjogren’s syndrome characterized by failure of kidney to secret hydrogen ions. This results in increased secretion of other cations including potassium with resultant metabolic acidosis and hypokalemia.

We present herewith a case of Sjogren’s syndrome with distal renal tubular acidosis who presented with a wide spectrum of neurological manifestations. Further evaluation of this patient along with the brain imaging revealed osmotic demyelination. As patient did not have hyponatremia at any point during hospitalization, we could attribute occurrence of Osmotic demyelination syndrome to hypokalemia only.