BACKGROUND: Repair of the rare median cleft lip occurring with agenesis of the columella is a challenge as there is a major deficiency of skin and underlying structures. MATERIAL AND METHODS: Over a 23- year period, five children underwent construction of a columella. A new surgical technique was designed for this: an internal dorsal nasal flap was used to create a cutaneous-cartilaginous flap inside the nasal dome, and was then sutured inferiorly against a superior triangle of the repaired median cleft lip. RESULTS:Projective nasal growth could be seen in three of the five surgically constructed columellae in whom the medium-term results could be evaluated. CONCLUSION:Five holoprosencephaly cases, all with a median cleft lip and agenesis of the columella, underwent a median cleft lip repair and the creation of a columella by means of an internal nasal dorsal skin flap procedure.