Double lip is a rare anomaly that may be congenital or acquired and affects upper lip more commonly than the lower lip. This deformity consists of an accessory fold of redundant mucous membrane inside the vermilion border. It affects esthetics and may also interfere with speech and mastication. Simple surgical excision produces good functional and cosmetic results. We describe a case of congenital maxillary double lip along with a brief review of the literature and discuss the differential diagnosis. This anomaly is of more relevance in dentistry as a general dental practitioner is usually, the first professional to recognize and establish the diagnosis.