Lesch-Nyhan syndrome is a rare X-linked recessive disorder characterized by a deficiency of the enzyme hypoxanthine-guanine phosphoribosyl transferase, which is important in the metabolism of purines. Prevalence of LeschNyhan syndrome is 1:100,000 to 1:380,000 live births, and Lesch-Nyhan syndrome mainly affects males. It is characterized by neurologic dysfunction, uric acid overproduction, and cognitive and behavioral disturbances. The selfmutilation is the most distinctive symptom in Lesch-Nyhan syndrome, appearing about 1 year of age. It is expressed as persistent bites in the oral mucosa, tongue, lips, and fingers, which leads to the total or partial destruction of the affected tissues. Several medical and dental management methods might be performed in Lesch-Nyhan syndrome patients. Dental approaches to prevent injuries caused by self-mutilation include treatments with oral appliances such as mouth guards or lip bumpers, extraction, and orthognathic surgery. This report described two brothers with Lesch-Nyhan syndrome, who injured themselves on oral tissues. The methods of tooth reduction and extraction were performed to prevent injuries caused by self-mutilation.

Ameloblastic fibro-odontoma (AFO) is a rare odontogenic tumor, which occurs in young children before the age of 20 years. Radiologically, it is a well-defined radiolucent lesion containing radiopaque masses. Histopathologically, AFO is composed of odontogenic epithelium in a primitive-appearing connective tissue and hard tissue consisting of enamel and dentin. It is commonly found in the mandibular posterior region. AFO may be asymptomatic and is often associated with delayed tooth eruption. As it shows similar characteristics clinically and radiologically to odontoma, differential diagnosis through histopathological examination is important. Treatment of AFO is conservative enucleation, and teeth enclosed or associated with the lesion may require extraction. In this report, 2 young patients who visited our clinic with a chief complaint of delayed eruption were diagnosed as AFO with radiological and histopathological examination. After the surgery, the healing status was found to be favorable, and no evidence of recurrence was observed.