Abstract: Tubulointerstitial nephritis and uveitis (TINU) syndrome is defined as the occurrence of tubulointerstitial nephritis (TIN) and uveitis. According to present epidemical statistics, women are more likely to be affected, especially in children and adolescents. The sequence of the clinical manifestations on oculus and kidney may be successive or simultaneous. Humoral immunity and cellular immunity play an important role in this immunological disorder with the reference of HLA gene family. Environmental triggers are also the possible causes of this syndrome. At the beginning of this syndrome, clinical manifestation would be dramatically insidious, with fever, rash, arthralgia, abdominal pain and weight loss. The typical renal manifestation is unexplained renal insufficiency caused by immune-mediated infiltration of renal tubulointerstitial inflammatory cells. The Ocular manifestation is specialized as bilateral acute anterior uveitis. It is an exclusive diagnosis in this syndrome. The infectious uveitis and other systemic and autoimmune diseases caused by bilateral uveitis should be excluded. High heterogeneity has been described in the sequence of uveitis and interstitial nephritis. The challenges in diagnosing this syndrome lie in the long-time interval between the oculus and the kidney and it ' s hard to figure out which organ is affected when systematic therapeutic methods have been used in the one. Furthermore, there is no laboratory examination that can differentiate this syndrome. The process and criticism of diagnosing this syndrome has been in debated yet. According to present reports, partial and systematic steroid therapy has been shown to be effective. From a prognostic point of view, acute tubulointerstitial nephritis a self-limited disease, and the risk of developing chronic kidney disease in the adult population should be taken into account. In contrast, uveitis, especially in children, can persist or recur.