BACKGROUND: Tessier described rare craniofacial clefts anatomically. The no. 7 cleft is a lateral facial cleft consisting of macrostomia, lateral facial muscular diastasis, and bony abnormalities of the maxilla and zygoma. Early computed tomographic imaging provided preliminary insight into the bony abnormality. This article reviews this patient group, defining the clinical and radiological features, to advise optimal protocol management. METHODS: A retrospective case-note review of 15 patients with Tessier no. 7 clefts managed by the Australian Craniofacial Unit over the past 25 years was performed. Cases of hemifacial microsomia and Treacher-Collins syndrome were excluded. Clinical features of the patient group were analyzed with photography (all clefts) and imaging (seven clefts). Surgical management and outcome are reviewed. RESULTS: Fifteen patients and 18 clefts (three bilateral) were treated during the time period of the study. All patients had macrostomia (mean length, 2 cm) and 94 percent had soft-tissue ridging from muscular diastasis directed toward the tragus (44 percent), temporal area (28 percent), or lateral canthus (22 percent). Bony abnormalities included simple clefting of the maxillary molar region in 55 percent, maxillary duplication in 39 percent, and intermaxillary fusion in 6 percent. Surgical intervention included macrostomia repair at the first available opportunity, resection of maxillary duplication (median age, 4 years), and alveolar bone grafting to the cleft (median age, 10 years). Optimal follow-up of these patients is impaired because of long distances required for review. Patients treated with this management protocol have had good functional and aesthetic results to date. CONCLUSIONS: This article describes the Tessier no. 7 cleft in great detail and suggests a new subclassification of the bony abnormality. An adjusted management protocol is proposed to address the bony abnormalities of cleft and duplication, with favorable outcomes for treated patients with this condition.