Complete or partial lip clefts in the midline of the upper lip are defined as median clefts of the upper lip. In 1974, Tessier numbered the craniofacial clefts from 0 to 14 counterclockwise in a study involving 336 patients. Upper lip midline clefts are named Tessier 0 clefts. Despite the diversity in classifications, upper lip midline cleft is rare. Its incidence among cleft patients varies between 0.43% and 0.73%. If the more common forms of cleft lip and palate are not taken into account, the incidence of atypical cleft is estimated at 1/105. A median upper lip cleft can be defined as a cleft that passes vertically through the midline of the upper lip. These clefts occur due to incomplete or complete nondevelopment of the medial nasal prominences. In this study, the authors shared a case diagnosed prenatally and referred to their clinic for cleft lip repair.