BACKGROUND:Keratoacanthoma (KA) is a rapidly growing epithelial tumour with histopathologic and clinical features similar to squamous cell carcinoma (SCC) and a certain tendency toward spontaneous regression.CASE PRESENTATION:This article presents a unique and rare case of keratoacanthoma arising from the upper lip of a young male patient. These two features are in contrast to most of the reported cases in elder male individuals and on the lower lip. Relevant management protocol of the case has also been discussed.CONCLUSION:The article emphasises the significance of discerning such lesions from squamous cell carcinoma thus carrying diagnostic and therapeutic implications. However, in case of the dilemma it is prudent to assume that the lesion is SCC unless proved otherwise clinically and histologically.