Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder that is present from birth and can impact various organs and tissues in the body. It arises from abnormalities in genes, primarily on chromosome 11. One of the possible associated features of BWS is the presence of a preauricular sinus, also known as a congenital ear fistula, and can be linked to serious conditions involving kidney dysfuntion. Although the precise relationship between BWS and preauricular malformation is not fully understood yet, research has revealed that the condition affects the regulation of genes that play a crucial role in growth and development during embryogenesis.