BACKGROUND: VACTERL association is diagnosed based on the non-random co-occurrence of at least three out of six congenital malformations. These include vertebral defects, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without oesophageal atresia, renal malformations, and limb defects. There is no known link between VACTREL association and metopic synostosis in the literature. The authors describe the co-occurrence of VACTERL association and metopic synostosis in three cases. RESULTS: There were 122 operated cases of metopic synostosis at our institution from 1999 to 2023, with a 2.3:1 male-to-female ratio. All three cases of VACTERL association and metopic synostosis co-occurred in female patients. The female predominance observed is not seen in either VACTERL association or metopic synostosis, and the absence of renal anomalies in two patients is unusual. Genetic testing did not identify any variants of clinical significance in all three cases. Given that VACTERL association is a diagnosis of exclusion, other rare syndromes were considered but ultimately excluded. CONCLUSION: Based on the published prevalence of VACTERL association and metopic synostosis, the odds of a random co-occurrence would be incredibly rare. This raises the possibility of a common aetiology, potentially in the form of an underlying pathogenic variant that cannot yet be identified with current gene panels.