Climatic droplet keratopathy (CDK) is an acquired and potentially handicapping corneal degenerative disease that is highly prevalent in certain rural communities around the world. It predominantly affects males in their 40’s and is known by many other names such as Bietti’s band-shaped nodular dystrophy, Labrador keratopathy, spheroidal degeneration, chronic actinic keratopathy, oil droplet degeneration, elastoid degeneration and keratinoid corneal degeneration. CDK is characterised by the haziness and opalescence of the cornea’s most anterior layers, which go through three stages with increasing severity. Globular deposits of different sizes may be histopathologically observed under the corneal epithelium by means of light and electron microscopy. The coalescence and increased volume of these spherules may cause the disruption of Bowman’s membrane and the elevation and thinning of the corneal epithelium. The exact aetiology and pathogenesis of CDK are unknown, but they are possibly multifactorial. The only treatment in CDK advanced cases is a corneal transplantation, which in the impoverished regions of the world is not an available option. This review highlights new scientific evidence of the expanding knowledge on CDK’s pathogenesis, which will open the prospect for new therapeutic interventions.