Median facial dysplasia (MFD) is a distinct and unique disorder of the craniofacial region that is characteristic of deficient mid facial structures with the addition of a unilateral or bilateral cleft lip with or without a cleft palate. A cleft lip which is associated with MFD whether it is unilateral or bilateral does not represent a typical cleft lip and poses some challenges in reconstruction. The management of such cleft lip has been rarely discussed in previous literatures; the main obstacle comes in identifying the anatomical landmarks to establish an esthetic reconstruction of the lip. However, reasonably good result can be achieved when the adjacent and distant anatomical structures are correlated to aid the reconstruction.