The giant keratoacanthoma (KA) is a rare variant of KA with a maximum size exceeding 2–3 cm. Like other forms of KA, it has a tendency to spontaneously regress but can cause significant anatomic damage. A 69-year-old male presented to our hospital with a giant KA of the nose that showed complete pathological regression by the time of surgery. Pathology showed dermal scar with keratin granulomas extending through the nasal wall to the respiratory mucosa. A total of 57 similar cases from the English literature were reviewed for comparison. Few provide similar details of histological regression. Literature cases occurred predominately in males (74.1%) with a mean age of 59 years. Head tumors were most common (70.7%) and most were treated by surgery (34.5%) or a combination of surgery and radiotherapy (24.1%). Other treatment modalities reported include methotrexate, 5-fluorouracil, and interferon.