Background: Children with myelomeningocele (MMC) develop a wide variety of hip deformities such as muscle imbalance, contracture, subluxation, and dislocation. Various methods and indications have been introduced for treatment of muscle imbalances and other hip problems in patients with MMC but there is no study or meta-analysis to compare the results and complications. This review aims to find the most acceptable approach to hip problems in patients with MMC. Methods: MEDLINE was searched up to April 2015. All study designs that reported on the outcomes of hip problems in MMC were included. From 270 screened citations, 55 were strictly focused on hip problem in MMC were selected and reviewed. Results: Complex osseous and soft tissue reconstructive procedures to correct hip dysplasia and muscle balancing around the hip are rarely indicated for MMC patients without good quadriceps power. Conclusion: Over the years a consensus on the best algorithm for treatment of hip dislocation in myelomeningocele has been missing, however, muscular balancing with/out osseous procedure seems a reasonable approach especially in unilateral mid-lumbar MMC.