Keratoacanthoma (KA) is a common but underreported tumor of the skin. Two striking features of KA are its clinical behavior with spontaneous regression after rapid growth and its nosological position on the border between benignity and malignancy. We review current knowledge on the clinical, histopathological, and dermoscopic features of KA to ensure a proper diagnosis and describe its variants, including different types of multiple KAs. We highlight current concepts of KA ethiopathogenesis with special emphasis on the genetic background of multiple familial KA, the role of Wnt signaling pathway, and induction of KA by BRAF inhibitors and procedures of esthetic dermatology. Finally, treatment strategies are presented with surgical excision as a first option, followed by other modalities, including intralesional chemotherapy, topical and systemic agents, lasers, cryotherapy, and photodynamic therapy.