Butterfly spine is a rare benign congenital abnormality. The onset of a minimum of three of the congenital malformations of vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities often characterises the VATER/VACTERL relationship. Recognising this anomaly is crucial for diagnosis, although this rare aberration is thought to be asymptomatic most of the time. Here we are describing a case of a one-year-old female child who has tetralogy of Fallot, congenital anal atresia, vesicovaginal fistula, and butterfly vertebrae which were found as an incidental finding. Furthermore we suggest screening all the children with any one abnormality of VACTERL, usually vertebral anomalies are screened.