Linear porokeratosis is a rare subtype of porokeratosis with a higher rate of malignant transformation than other subtypes of porokeratosis. Identification of cornoid lamellae on histology allows for definitive diagnosis, which makes high clinical suspicion and appropriate biopsy essential in establishing the correct diagnosis and developing an effective management plan. We present a case report of linear porokeratosis and discuss aspects of etiology, diagnosis, and management.