Microscopic colitis (MC) is a chronic inflammatory disease of colon with clinical presentations of chronic, watery, nonbloody diarrhea, and normal or almost normal endoscopic findings. Confirmation of a diagnosis of MC requires microscopic examination on colon biopsy to identify characteristic morphological features, in which 2 main subtypes of MC, lymphocytic colitis (LC) and collagenous colitis (CC), have been described. Although the pathogenesis of MC is still unclear, studies have revealed associations of MC with many risk factors and other diseases such as celiac disease, inflammatory bowel disease, and medication use. Meanwhile, variants of MC, MC incomplete, or MC-like changes in other conditions are still diagnostic dilemmas for pathologists. The goal of this paper is to systemically introduce the clinicopathologic features of MC and focus on unusual features of MC and its associations with other conditions.