Ankle replacement in the presence of a varus deformity is an evolving field. Although the initial results were disappointing, numerous advances in the understanding of the condition and operative techniques have been made. More recent reports show good short-term results, especially when adjunctive procedures are combined, not only to achieve a neutral alignment but also the restore lateral ligamentous stability. As a result, it is possible to correct varus deformities of 20° or more with ankle replacement. It is likely that, with a reliable correction of alignment and balance, that the long-term results of ankle replacement in significant varus deformity will be promising.