Median cleft lip is a midline vertical cleft through the upper lip in the absence of a prolabial remnant. This may occur as a sporadic event or be part of an inherited sequence of anomalies. A failure of formation or fusion of the medial nasal prominences derived from the frontonasal prominence is ultimately responsible for this aberration. Two categories of dysplasia are associated: (1) frontonasal deformity associated with hypotelorism and (2) median facial cleft syndrome associated with hypertelorism. A patient presents with median cleft lip, mild bifid nose, and hypertelorism. Following surgical reconstruction, a good result is achieved. The embryology, implications for associated abnormalities, and surgical technique for treating these cases are discussed.