Porokeratosis is an epidermal differentiation disorder caused by germline pathogenic variants in mevalonate pathway genes, with a second-hit mutation in lesional skin. Many with germline variants have limited disease and may not seek care. Porokeratosis lesions are frequently mistaken for benign conditions, contributing to underdiagnosis. A significant and underrecognized comorbidity of porokeratosis is its association with skin cancer.