Microscopic colitis is an inflammatory bowel disease that commonly presents with debilitating chronic watery diarrhea. Recent epidemiologic studies and randomized trials of therapeutics have improved the understanding of the disease. Medications, such as nonsteroidal anti-inflammatories, proton pump inhibitors, and antidepressants, have traditionally been considered as the main risk factors for microscopic colitis. However, recent studies have challenged this observation. Additionally, several epidemiologic studies have identified other risk factors for the disease including older age, female sex, smoking, alcohol use, immune-mediated diseases, and select gastrointestinal infections. The diagnosis of microscopic colitis requires histologic assessment of colon biopsies with findings including increased in intraepithelial lymphocytes with or without expansion of the subepithelial collagen band. The pathophysiology is poorly understood but is thought to be related to an aberrant immune response to the luminal microenvironment in genetically susceptible individuals. Antidiarrheal medications, such as loperamide or bismuth subsalicylate, may be sufficient in patients with mild symptoms. In patients with more severe symptoms, treatment with budesonide is recommended. Maintenance therapy is often necessary and several potential treatment strategies are available. Biologic and small molecule treatments seem to be effective in patients who have failed budesonide. There is an unmet need to further define the pathophysiology of microscopic colitis. Additionally, trials with novel therapies, particularly in patients with budesonide-refractory disease, are needed.