Acute tubulointerstitial nephritis and bilateral uveitis (TINU) syndrome is a rare disease usually occurring in young women. We report the exceptional case of a 48-year-old man with TINU syndrome who had a 10-month history of interstitial nephritis before the onset of uveitis. Findings from the renal biopsy specimen indicated tubular atrophy, dense infiltration of lymphocytes, granulomatous infiltration with multinucleated giant cells, and disruption of the vessel wall. The patient was initially diagnosed to have chronic kidney disease; interstitial nephritis could not be diagnosed until blurred vision occurred. However, he was successfully treated with steroid pulse and oral maintenance therapy. Steroid therapy may be effective to treat TINU syndrome irrespective of the onset of the disease.