Abstract
INTRODUCTION: Midline cleft of mandible, classified as Tessier 30 clefts is extremely rare, with less than 100 reported cases in the latest studies. Variations in severity and associated malformations have been reported before. CASE PRESENTATION: In this report, we present the first documented Iranian case of Tessier 30 with median cleft of lower lip and bifid tongue concomitant with congenital heart defects. CONCLUSIONS: We explain embryologic origin, differential diagnosis, other associated anomalies and its treatment by reviewing literature.