The physiopathogenesis of adolescent idiopathic scoliosis remains unknown. However, a multifactorial pathogenesis is being assumed. Besides biomechanical, biochemical, and genetic factors, some studies have focused on congenital or acquired abnormalities in the vestibular organ with consecutive development of scoliosis. This study aims to analyze a possible correlation between any vestibular organ congenital or acquired pathologies and scoliosis based on the current literature. Therefore, we conducted a literature search in three databases, with search terms such as “scoliosis,” “organ of balance,” “idiopathic scoliosis,” “vestibular organ,” “spine,” and “balance.” Fifteen studies were selected and used for research. The relationship between scoliosis and vestibular organ abnormalities was recorded from all included works. Seven studies demonstrated a direct correlation between vestibular organ anatomical abnormalities and the form of the scoliotic spine. Another study confirmed the influence of the pathology of the vestibular organ on scoliosis but questioned whether it had an impact on the formation or the progression of the curvature. Others demonstrated a temporal overlap of the embryonic development of the vestibular organ and the beginning of pre-scoliotic characteristics, but their relationship remained questionable. In three studies, the correlation remained unclear, and any context has been denied. It seems unlikely that an isolated vestibular disorder can trigger structural scoliosis. However, the vestibular system pathologies may certainly occur in the multifactorial genesis of idiopathic scoliosis. Whether the correlation refers to the expression or the progression of scoliosis or may even have an influence on both remains unclear. New treatment options could be derived from these findings with a positive influence on the course of the deformity.