Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare and poorly understood condition that is likely underdiagnosed. The possibility that uveitis and acute tubulointerstitial nephritis do not occur simultaneously may make diagnosis more difficult. Treatment consists of systemic corticosteroids and potentially non-steroid immunosuppressants. Renal disease usually resolves spontaneously or with appropriate treatment, however, ocular manifestations may be chronic or relapsing. We report a case of tubulointerstitial nephritis and uveitis syndrome in a 12-year-old female.