This study aimed to investigate the clinical characteristics according to Tessier classification and evaluate the surgical outcomes in patients with rare craniofacial cleft (RCC) primary repair. A retrospective study on 30 patients with RCC was conducted at the Department of Craniofacial and Plastic Surgery of the Vietnam National Hospital of Pediatrics. Rare craniofacial cleft was recorded according to Tessier's classification and was analyzed for gender, affected side, clinical characteristics, and associated abnormalities. Primary surgical correction was based on the concept of aesthetic units with multiple Z-plasty. Preoperative deformities and postoperative outcomes were evaluated with anthropometric measurements using the Versnel scoring system. The most common type was Tessier 7 cleft (T7), followed by T0. There was no difference between the frequency of males and females. Patients with unilateral cleft accounted for a larger proportion than bilateral cleft (76.5% versus 23.5%
P = 0.029 <
0.05). The median and paramedian cleft groups (T0, T1, T30) affected orbit 22.2%-nose 77.8%-mouth 44.4%. The oblique clefts (T3, T4, T5, T11) affected orbit 100%-nose 50%-mouth 50%. The transverse cleft group (T6, T7, T8) affected mouth 94.1%-ear 29.4%. Rare craniofacial cleft may present alone or in a syndrome (Treacher Collin, Goldenhar, Hemifacial atrophy), or in combination with other abnormalities. Repairing RCC with aesthetic units with multiple Z-plasty has improved facial balance and restored key landmarks with acceptable scar position. The Versnel scoring system can serve as an objective instrument to measure the surgical outcomes of RCC repair and can be used to evaluate the influence of growth.