Xanthogranulomatous inflammation is an unusual destructive chronic inflammatory process that involves various organs & tissues particularly gallbladder (xanthogranulomatous cholecystitis) & kidney (xanthogranulomatous pyelonephritis). Xanthogranulomatous appendicitis (XA) is a rare form of appendiceal inflammation that has been regarded as an unusual healing pattern of appendicitis associated with delayed or interval appendectomy. It poses a significant diagnostic challenge because it can mimic clinically, radiologically, & even pathologically malignant tumors as well as other inflammatory processes of appendix. Little information has been written in the literature regarding this entity. In this review article, data have been collected from all the previously reported cases in the literature including my observation in a XA case I have diagnosed.