<p>The median cleft of the upper lip is rare. The isolated vermilion location with a normal cupid’s bow was never described in the literature. This article aims to discuss its embryological origin, allowing its distinction from other entities of midfacial malformation, such as those seen in holoprosencephaly, and to describe an innovative repair technique using a double split-thickness myo-mucosal vermilion flap to achieve a high level of aesthetic result. This technique was used in patients presenting with a median vermilion cleft without any neurological impairment, thereby eliminating the need for further neurological exploration. There were no postoperative complaints, and labial continuity was achieved with a natural projection of the tubercle and well-concealed scars. We emphasize that the median cleft of the upper vermilion represents a distinct pathological entity that differs from those seen in the midfacial malformation associated with holoprosencephaly. This type of cleft does not present any incompatibility of life. Compared with a Goldstein flap, the double split-thickness myo-mucosal flap is less invasive, based on the superficial vascular network, leaving the mucosa free of scars.</p>