Polyvinylpyrrolidone (PVP), a polymer of the monomer N-vinylpyrrolidone with various molecular weights, was originally developed as a plasma expander. Currently, it is widely used in hair sprays, skin care products, fruit juices, and as a retarding agent in drugs such as procaine and hormones. PVP polymers with a molecular weight greater than 20,000 cannot be excreted by the kidneys and therefore are phagocytosed and permanently stored in the reticular endothelial system, leading to the so-called PVP storage disease. We report a case of localized cutaneous PVP storage disease presenting with persistent upper lip swelling and mimicking cheilitis granulomatosa, which has never been reported before.