Branchio-Oto-Renal Syndrome (BOR) is a rare genetic disorder characterised by abnormal development of the gills, ears, and kidneys. This syndrome can occur with various manifestations, but common symptoms include neck and facial structure abnormalities, hearing loss, outer ear abnormalities, and kidney problems. BOR has multiple genetic forms, and in some cases, a correlation between Branchio-Oto-Renal Syndrome and preauricular sinus has been observed. This association can be explained by the fact that both conditions stem from abnormalities during the embryonic development of neck and ear structures. Specifically, alterations in the branchial ridges during fetal development can cause the formation of the preauricular sinus and the manifestation of the defects associated with BOR.