Abstract: Cronkhite-Canada syndrome (CCS) is a rare non-hereditary disease characterized by multiple gastrointestinal polyps and ectodermal abnormalities. Its etiology remains unclear, and there is a lack of a gold standard for diagnosis. Patients with CCS have a higher risk of developing gastrointestinal tumors compared to the general population. Early diagnosis is challenging, and the disease is prone to recurrence, necessitating long-term follow-up. The Digestive Disease Branch of China Alliance for Rare Diseases/Beijing Association of Rare Disease Diagnosis, Treatment and Security, in collaboration with the Rare Disease Collaborative Group of the Chinese Society of Gastroenterology, Chinese Medical Association, developed this consensus to provide corresponding recommendations on the pathogenesis, clinical manifestations, diagnosis, and treatment of CCS.