Unilateral vulva swelling during prepubertal period consists of a wide variety of inflammatory and neoplastic lesions. A group of labial lesions that showed normal radiologic, histopathologic and surgical findings are reported as childhood asymmetric labium majus enlargement. An 11-year-old girl with childhood asymmetric labium majus enlargement is presented to discuss the clinicopathologic features, differential diagnosis, and indications of conservative approach in the treatment of this rare entity.