Keratoacanthoma (KA) is a self-regressing cutaneous tumor sharing clinical and histopathological similarities with squamous cell carcinoma. It is relatively uncommon and presents diagnostic challenges, particularly in pediatric patients. This paper presents a case study of KA affecting the upper lip keratosis in a 6-year-old girl. Through a comprehensive analysis of the pathogenesis, differential diagnosis, and treatment approaches of KA in children based on existing literature, we aim to offer clinical insights and guidance for practice.