A keratoacanthoma is a rapidly growing cutaneous tumor that spontaneously involutes in most instances. A giant keratoacanthoma is a rare variant and are characterized by lesions larger than 20 mm in diameter. We report a 56-year-old man with a rapidly growing tumor of the right cheek, which was diagnosed as keratoacanthoma. The mass was excised completely under general anesthesia, followed by Limberg flap for reconstruction. Intraoperative frozen section histology suggested the lesion to be a well-differentiated squamous cell carcinoma, whereas final histopathology was consistent with keratoacanthoma. We herein report the first case of a giant keratoacanthoma treated with surgical excision in Korea and discuss the clinical and histopathological features of keratoacanthoma, with a review of the literature.