Aim: This case report aims to describe a rare congenital lesion of the incisive papilla with a high labial frenulum attachment, clinically mimicking congenital epulis but histopathologically diagnosed as an oral leiomyomatous hamartoma. Background: Oral leiomyomatous hamartoma is a very rare congenital lesion, mainly appearing on the median anterior maxilla/incisive papilla and tongue. Case description: This clinical paper is about a rare lesion in a 6-year-old female child whose parents reported to the department with the complaint of slow-growing soft tissue overgrowth between the front teeth of the upper jaw, present since birth. The soft tissue growth is now causing difficulty in biting food and is visible during smiling and speaking, causing an esthetically unpleasing appearance. The clinical examination also revealed a high labial frenulum attached to the lesion. The lesion was provisionally diagnosed as congenital epulis based on the clinical picture. However, after excisional biopsy and histologic evaluation with special stains, the lesion was finally diagnosed as leiomyomatous hamartoma. Conclusion: Surgical excision of the lesion followed by frenectomy was performed with no postoperative complications. Clinical significance: Owing to the rare occurrence and nature of mimicking congenital epulis, it is important for a dental practitioner to have knowledge about these types of lesions. The final diagnosis of such lesions can only be made after histopathological evaluation using special stains. This case report describes the clinical and histopathological features of a rare leiomyomatous hamartoma of the incisive papilla, along with high frenulum attachment and its management.