Median craniofacial dysplasia is a rare congenital anomaly with a broad spectrum of severity, which can be classified as hypoplasia, dysraphia, and hyperplasia, depending on the involved tissue amount. A retrospective chart review was performed of patients with median craniofacial dysplasia who underwent repair of the upper lip median cleft between January 2013 and February 2020. The median cleft of the upper lip was present in 5 cases. The average age at operation was 11 months. Two patients had a median notch in the vermilion, 2 patients had an incomplete median cleft lip, and 1 patient had a complete median cleft lip with the absence of columella, prolabium, and premaxilla. A variety of surgical correction was performed for each case, including simple rhombus-shaped excision, modified version of straight-line repair, and columella reconstruction using an intranasal dorsal flap and bilateral cleft margin flaps. Each case needs to be carefully assessed with individualization for appropriate surgical treatment.