Keloid disorder (KD) is an inherited wound healing ailment, frequently seen among Africans /African Americans and Asians. Genetics of this disorder continues to be obscure and poorly understood. Clinical manifestation of KD is quite variable and very diverse, spanning from individuals with one or very few small keloidal lesions, to those with numerous and very large lesions covering large portion of their skin. Ears are common locations for development of keloids. Ear piercing is by far the leading triggering factor for ear keloid formation in genetically predisposed individuals. Although there are numerous publications about ear and earlobe keloids, there is a void in medical literature about massive ear keloids. This paper focuses on the natural history of massive ear keloids and risk factors that lead to formation of these life-changing and debilitating tumors and recommendations for prevention.