
Ameloblastoma is a most common benign odontogenic neoplasm which effects the maxillofacial region and more frequently affecting the mandible. It exhibits various clinical, radiological and histopathological features based on different types of ameloblastoma. Among these types, unicystic ameloblastoma (UA) is the least seen variant of ameloblastoma, with good prognosis and less recurrence if detected at the earliest. UA refers to those cystic lesions that show clinical, radiographic, or gross features of a jaw cyst but on histological examination show a typical ameloblastomatous epithelium lining the cystic cavity, with or without luminal and/or mural tumour proliferation. UA commonly occurs in 2nd and 3rd decades of life but only about 10% of cases are reported in children and less than one third of those occur in children below 10 years. We report a rare case of 5 year old male patient, presenting with a swelling in the left lower jaw region which was diagnosed as UA by correlating the clinical, radiological, and histopathological findings. Thus early diagnosis of UA in children is mandatory as it affects the growth of the jaw. The incidence rate, behavior and prognosis of the tumour in children make the surgical consideration different from adults.