Introduction: Nephrocalcinosis is a rare complication of chronic tubulointerstitial nephritis observed in primary Sjögren's syndrome. It can precede subjective sicca symptoms.
Observation: We report the case of a 50-year-old woman who presented with a primary Sjögren's syndrome. The first symptoms appeared 10-years-ago while she was affected with a nephrocalcinosis.
Conclusion: Autoimmune investigations for Sjögren's syndrome should be initiated in any patient presenting with nephrocalcinosis and distal renal tubular acidosis.