A 27-year-old male was referred to a Brazilian School of Dentistry, in November 2016, complaining about a slowly progressive asymptomatic swelling on the right side of the posterior mandible. The medical history was unremarkable. Upon intraoral examination, the right lower buccal vestibule was obliterated due to the expansion of the buccal cortical plate. The extraoral examination showed slight facial asymmetry. Panoramic radiographs showed a large unilocular radiolucent lesion with well-defined borders involving the unerupted 48 teeth. An incisional biopsy was performed. Microscopically, hematoxylin and eosin sections presented strands, cords, and nests of odontogenic epithelium supported by a primitive-looking myxoid connective tissue, resembling the dental papilla. The islands showed a peripheral rim of columnar similar to the inner enamel epithelium. The center of the islands revealed a loosely arranged spindle-shaped epithelium identical to stellate reticulum. The patient is undergoing regular clinical and radiographic follow-up; after 6 years, no recurrence was observed. A 27-year-old male was referred to a Brazilian School of Dentistry, in November 2016, complaining about a slowly progressive asymptomatic swelling on the right side of the posterior mandible. The medical history was unremarkable. Upon intraoral examination, the right lower buccal vestibule was obliterated due to the expansion of the buccal cortical plate. The extraoral examination showed slight facial asymmetry. Panoramic radiographs showed a large unilocular radiolucent lesion with well-defined borders involving the unerupted 48 teeth. An incisional biopsy was performed. Microscopically, hematoxylin and eosin sections presented strands, cords, and nests of odontogenic epithelium supported by a primitive-looking myxoid connective tissue, resembling the dental papilla. The islands showed a peripheral rim of columnar similar to the inner enamel epithelium. The center of the islands revealed a loosely arranged spindle-shaped epithelium identical to stellate reticulum. The patient is undergoing regular clinical and radiographic follow-up; after 6 years, no recurrence was observed.