Acute tubulointerstitial nephritis (ATIN) is a unique form of interstitial nephritis that is responsible for approximately 15% of the cases with renal failure. Mostly, ATIN begin when certain medications, infections (such as Chlamydia and Epstein Barr virus), autoimmune diseases, and other factors cause an immune-mediated tubulointerstitial injury. Patients often present with fever, rash, dehydration, enlarged kidneys, uveitis, and arthralgias. The median age of patients with ATIN is found to be 15 years and most patients are adolescents and young women. The disease affects females more than males with the ratio 2-3:1. The best diagnostic method for ATIN is renal biopsy examination. Corticosteroids have provided some benefits to the patients; however, nephrectomy would be the only choice in advanced cases.