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Background: Cherubism is an uncommon hereditary benign fibro-osseous disorder characterized by bilateral enlargement of the mandible and the maxilla that presents with varying degrees of involvement and a tendency toward spontaneous remission. On radiography cherubic lesions appear as cystic multilocular radiolucencies limited to the jaw bones. Case Report: A 5-year-old boy was referred to the Department of Maxillo-Facial Surgery due to deformation of the lower and middle section of the face and displacement or absence of teeth. A panoramic radiograph and a computed tomography revealed extensive multilocular, bilateral radiolucent areas and marked bony expansion in the mandible and maxilla, with sparing of the mandibular condyles. Histopathological evaluation of an incisional biopsy of the left maxilla and genotypic characterization confirmed the diagnosis of cherubism. Conclusions: The radiologic characteristics of cherubism are not pathognomonic but the diagnosis is strongly suggested by bilateral relatively symmetric jaw involvement that is limited to the jaw bones and, together with clinical and histopathologic findings, enables the diagnosis of cherubism. Genotypic characterization confirms the diagnosis.