Omphalocele is a congenital abdominal wall defect that occurs approximately 1 in 4000-6000 live births. The abdominal-visceral disproportion, large diameter of the defect, volume of liver in the sac along with high incidence of associated anomalies make the surgical management a real challenge. Currently, there are two strategies for managing giant omphaloceles, staged surgical closure and nonoperative delayed closure. The combined treatment with PPP and BoNT/A injection has recently been described in adults. There is strong evidence on safety and efficacy of the use of BoNT/As in other areas of pediatrics and no recent reports of PPP use in children. Also, there are no data available about the combination of both techniques in pediatric population. The purpose of this manuscript is to report a case of a 7-year-old female child that was referred to our institution with a large ventral hernia secondary to omphalocele. We opted for a combined approach with BoNT/A injection and PPP before the definitive surgery. The surgical result was great with midline closure with no tension and no need for prosthetic substitution or component separation needed. To our knowledge, this is the first case report of BoNT/A injection and PPP for large ventral hernias in children. BoNT/A application was safe and the PPP technique was also proved to be applicable on children. We believe that the combination of BoNT/A and PPP presented to be a safe approach with an excellent result, particularly for not needing abdominal wall prosthetic substitution.