Median cleft lip is a rare congenital anomaly. It may appear in the upper or in the lower lip ranging from a lip fissure to a complete cleft with or without brain deformity. Early surgical correction is indicated even when minimal expression of the deformity is present. A six week old baby was presented with a minimal expression of a median cleft in the upper lip. Clinically, it occured as a skin insertion in the midline of the vermilion. In the follow-up there occured a gap in the muscle midline and a midline diastema. At the age of 12 month the surgical correction was performed. Intraoperatively a dermoid cyst was seen, which caused a gap in the orbicularis oris muscle and a midline cleft of the intermaxillary bone. Palabras clave: Dermoidzyste, mediane Lippenspalte, Diastema mediale, Lippenfissur