VACTERL Association is an acronym for vertebral defects, anal atresia, cardiac anomalies, jolt-esophageal fistula, renal anomalies, and limb. The etiology is uncertain and there is no apparent relationship with chromosomal alterations; presenting rare and variable way. Its incidence is estimated at 1 in every 10,000 births, being higher in children relationship 2, 6: 1. The case of a newborn preterm (30 weeks), is presented with four of the seven diagnostic criteria for VACTERL. Physical examination was appreciated hypoplasia of the first finger of left hand, by impossibility of step of probe og, is diagnosed esophageal atresia, radiologic study showed Jolt-esophageal fistula and presence of hemivertebra at lumbar level; Echocardiogram detected foramen oval permeable and persistent patent ductus arteriosus of 2,5 mm. This entity, with relative rarity in terms of its presentation, expresses broad conditions that vary from one individual to another, generating easily errors in diagnosis and clinical approach, increasing mortality in newborn infants.