Dermoid cyst can also be seen head and neck region although it is generally seen in testis and ovary. The most common location in the head and neck region is the external third of the eyebrow. Sublingual location is very rare. Surgical treatment is by enucleation via in-traoral or extraoral approach. We present herein a case of giant sublingual dermoid cyst, origin from the mandibular sym-phisis is by enuclation via different approach that intraoral and extraoral approach are com-bined and reviewed the relevant literature.