Background Mucus protects the epithelium against invaders and toxic materials. Sticky and thick mucus is characteristic of CF. Objective The aim of this systematic review is to characterize the specific mucins secreted in the lung and intestinal tract of CF patients. Methods A systematic literature search was conducted up to 31.12.2019. The following terms were used: “cystic fibrosis” AND “mucin”. Case control studies comparing mucin expression in CF patients to healthy controls were included. Results We found 741 eligible studies, 694 studies rejected because they were performed in animals and not in full text, and 32 studies were excluded being editorials, duplications, review articles, meta-analysis, or not in English. Fifteen studies were eligible for our study, including 150 CF patients compared to 82 healthy controls all fulfilled the inclusion criteria. The main mucin types expressed in the sinus sub-mucosal glands, sputum, trachea-bronchial surface epithelium and lung sub -mucosal glands were MUC5AC and MUC5B. Increase in the number of sinusoidal sub-mucosal glands and expression of MUC5B was found in CF patients, but no such difference from healthy controls was found for the number of goblet cells in the surface epithelium nor in the expression of MUC5AC. The opposite was found in the trachea-bronchial surface epithelium and in the lungs. Conclusions Increased expression of MUC5AC in the surface epithelium and of MUC5B in the sub-epithelial glands may be the result of higher secretion rate of mucin into the lumen of the respiratory tract, causing mucus plaque, infection and inflammation.© 2020 S. Karger AG, Basel.