Porokeratosis is a group of keratinization disorders with heterogeneous clinical presentations, characterized by annular plaques with hyperkeratotic rims known as cornoid lamellae. Genetic mutations, particularly in the mevalonate pathway, underlie the disease, with environmental triggers like ultraviolet (UV) radiation and immunosuppression playing contributory roles. Several clinical forms exist, including disseminated superficial actinic porokeratosis (DSAP), linear porokeratosis, and porokeratosis of Mibelli. While typically benign, porokeratosis has a potential for malignant transformation, particularly into squamous cell carcinoma (SCC). This review delves into the pathogenesis, clinical features, diagnosis, and therapeutic options for porokeratosis, with a focus on emerging genetic findings.