Keratosis circumscripta, also known as psoriasis circumscripta with palmoplantar keratosis, is a rarely reported condition that manifests as well-circumscribed lesions consisting of grouped folliculocentric papules on the elbows, knees, neck, sacrum, posterior axillary folds, and hips. This condition typically begins in childhood and has been shown to improve, but not resolve, with keratinolytic therapies. Some debate exists concerning the terminology used to identify this condition. Our report contributes to this ongoing dialogue by presenting a case that supports recognizing keratosis circumscripta as a unique clinical entity. We describe the diagnosis and treatment of a boy with keratosis circumscripta. We also present a review of the literature pertaining to this condition and an overview of the controversy surrounding its terminology.