Once considered a rare disease among children of northern European decent with classical symptoms of malabsorption, celiac disease (CD) is now known to affect individuals in every decade of life and of many ethnicities and races. The current presentations of CD vary widely from an asymptomatic state to classical symptoms of abdominal pain, bloating, weight loss, diarrhea, and steatorrhea. They may also present along a spectrum with more subtle gastrointestinal and/or extraintestinal symptoms and signs of the disease. High-risk groups include family members of individuals with CD, as well as those with type 1 diabetes and a variety of autoimmune and other diseases. While biopsy remains the gold standard in diagnosis, serological tests are crucial in determining who should undergo endoscopy and biopsy. Currently, a gluten-free diet (GFD) is the only available therapy, and lifelong adherence to this diet is vitally important as continued gluten consumption may predispose patients to complications or associated disorders.