With the application of multimodal imaging techniques, a specific subtype of age-related macular degeneration (AMD),
known as cuticular drusen, has been identified. This subtype is closely associated with the progression of AMD, particularly non-exudative AMD, also referred to as dry AMD. However, there is a scarcity of literature focusing on the multimodal imaging characteristics of AMD combined with cuticular drusen. Therefore, studying and elucidating the multimodal imaging features of cuticular drusen, its differential diagnosis from other AMD subtypes of drusen, pathophysiological mechanisms, and treatment methods holds significant clinical importance. Multimodal imaging reveals cuticular drusenas numerous, symmetrical, evenly sized, yellow subretinal nodules in both eyes, exhibiting a typical "stars-in-the-sky" appearance on fluorescein angiography (FFA) and zigzag elevations between the retinal pigment epithelium (RPE) and Bruch's membrane on optical coherence tomography (OCT). The distribution characteristics of AMD patients with cuticular drusen vary greatly among individuals and can easily be confused with drusen from other AMD subtypes, so the differentiation from hard drusen, soft drusen, large colloidal drusen, and Sorsby's fundus dystrophy (SFD) is necessary. Studies suggest that cuticular drusen have a genetic, atherosclerosis- like pathogenesis which closely related to the accumulation of large lipoprotein particles secreted by the RPE. Future therapies targeting the complement system maybe employed to delay the progression of cuticulardrusen-related lesions. This article reviews the clinical manifestations, typical multimodal imaging features, differential diagnosis, genetic basis, pathophysiological mechanisms, complications, and clinical management strategies of cuticulardrusen.