Congenital superior oblique palsy in a patient with VACTERL association

Different types of ophthalmological defects have been reported to be accompanying vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies, and limb anomalies (VACTERL) association. A 7-year-old girl with a history of VACTERL association presented with upward drifting of the left eye and anomalous head posture to the right side and was diagnosed with congenital fourth nerve palsy. We report the first case representing a combination of congenital fourth nerve palsy with VACTERL association.