The aim of this review is to provide the readers current information on the etiology of clubfoot. Clubfoot etiology is currently still uncertain. We have not a satisfactory conclusion although different etiological factors multiple investigators found based on several studies. Studies have done by various points of view including histopathologic analyses, arrests in embryonic development, genetic background and syndromic associations. The general consensus is that histopathological findings will help to explain the persistence of clubfoot deformity and resistance to correction, if not the actual cause. Understanding the histopathological changes that characterize clubfoot and investigating adjunctive tools for follow-up of idiopathic clubfoot may be useful. Although the characteristic dysmorphic talar head and the dislocation of the navicular have never been observed at any stage of normal fetal development, embryonic development must be present from initial limb bud differentiation in an affected extremity. A genetic basis for isolated clubfoot is supported by various studies but none of them can be considered as an exact etiological factor. Clubfoot deformity may be associated with myelodysplasia, arthrogryposis, or multiple congenital abnormalities and syndromes but is most commonly an isolated birth defect and considered idiopathic. This article aims to provide insight by addressing all these subjects. Understanding the exact etiology of clubfoot may eventually be helpful for a classification so that both prognosis and appropriate treatment methods could be improved.