INTRODUCTION: Repair for giant incisional hernias is a challenge due to unacceptable high morbidity and recurrence rates. Several surgical techniques are available, but all are poorly documented. This systematic review was undertaken to evaluate the existing literature on repair for giant incisional hernia. METHODS: Literature was identified through a systematic search on PubMed, EMBASE, and CINAHL. We only included studies with clearly defined surgical techniques and defect size of at least 15 cm. The heterogeneity of the studies precluded a meta-analysis. The analysis was based on the premises of a systematic review of the literature. RESULTS: We identified 14 studies (1,198 patients) including one randomised trial. Studies were mainly small and retrospective and highly heterogenic regarding design, outcome, inclusion, and exclusion criteria. The overall morbidity rate was median 32 % with a wide range between studies of 4-100 %. The mortality ranged from 0 to 5 % (median 0 %) and recurrence rate ranged from 0 to 53 % (median 5 %). Study follow-up ranged from 15 to 97 months (median 36 months). Mesh repair should always be used for patients undergoing repair for a giant hernia, and the sublay position may have advantages over onlay positioning. To avoid tension, it may be advisable to use a mesh in combination with a component separation technique. Inlay positioning of the mesh and repair without a mesh should be avoided. CONCLUSIONS: Evidence to optimise repair for giant hernias is weak due to the heterogeneity and the poor quality of studies. However, sublay positioning of the mesh perhaps in combination with a component separation technique may be advantageous compared with other surgical techniques for giant hernia repair. Giant hernia repair is a challenging surgical procedure and severely lack evidence-based research from high-quality, large-scaled randomised studies.