This is a case report of an Odontogenic myxofibroma in a paediatric patient. This lesion, also called odontogenic myxoma, is an uncommon benign odontogenic tumour that derives from mesenchymal elements. Odontogenic myxofibroma in paediatric age is rare, because is more frequently discovered between the second and the fourth decade. Clinical, radiological and histological examinations were performed. Clinical examination showed a 3 cm painful swelling in the anterior mandible, and the panoramic radiograph confirmed the presence of an ovoid, well-definite radiotransparent area. Histologically, the odontogenic myxofibroma was characterized by an abundant mucoid stroma containing angular, spindle-shaped and rounded cells. The young age of the patient was considered an indication to perform a conservative approach, with the goal of avoiding the extraction of the elements interested. As reported in literature, the follow-up management depends on several variables, such as location, size, age of onset, and the type of surgical treatment. After 5 years, clinical and radiographic follow up showed no recurrence and the integrity of the anterior mandibular teeth.