Tubulointerstitial nephritis and uveitis (TINU) is a rare syndrome of unknown etiology involving the kidney and the eye. This is the first case reput of TINU in Korea. The diagnosis of TINU was confirmed in a 15-year-old girl on the basis of pathologically proven tubulointerstitial nephritis and bilateral uveitis. Although the renal symptoms disappeared spontaneously, uveitis showed only partial response to topical steroid treatment and persisted fir more than 11 months. TINU should be included in the differential diagnosis of tubulointerstitial nephritis of unknown etiology.