Bilateral Tessier no. 7 clefts are rarely reported in the literature. Here, we describe the presence of accessory maxilla with supernumerary teeth in a patient who exhibited bilateral Tessier no. 7 clefts; the diagnosis was established based on the patient\'s history, clinical presentation, and computed tomography images. A review of the available literature revealed 24 patients with Tessier no. 7 clefts from 2000 to 2020, including our patient. The most common clinical manifestation in patients with Tessier no. 7 clefts comprises bilateral facial clefts. Additionally, Tessier no. 7 clefts are more frequently found in boys or men, rather than in girls or women. The presence of an accessory maxilla with supernumerary teeth in a patient with bilateral Tessier no. 7 clefts is extremely rare. Early detection of craniofacial abnormalities is important, because it may influence patient prognosis and management.

A Tessier no. 7 cleft is a lateral facial cleft which originates from the oral cavity and extends towards the tragus, involving both soft-tissue and skeletal components. A male patient presenting with both maxillary jaw duplication and bilateral Tessier no. 7 clefts, which has been reported only twice in the literature, is described. Bilateral facial clefts, macrostomia and chondro-cutaneous remnants were noted, which were repaired and resected. With further growth, facial asymmetry and asymmetric facial nerve dysfunction became apparent. Radiographic examination showed an accessory maxillary jaw and a flattened and hypoplastic right coronoid process. A maxillary alveolar cleft was also present between the left second bicuspid and the second permanent molar. This case may represent an under-recognized phenotype with an unusual combination of maxillary jaw duplication, macrostomia, Tessier no. 7 clefts, and chondro-cutaneous remnants. A long-term follow-up of these patients is recommended as they often develop craniofacial deformities later in life.