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A 25‐year‐old male patient presented with swelling and pain in the mandibular anterior region for the previous 6 months. He had a history of repeated traumatic episodes associated with his lower incisors. A periapical radiograph revealed a radiolucent lesion involving the apices of the lower incisors, whereas cone beam computed tomography revealed a large osteolytic lesion involving all mandibular incisors and loss of labial and lingual cortical plates. Histological, immunohistochemical and bone scan examination of the curetted tissue established a differential diagnosis of spindle cell rhabdomyosarcoma of the anterior mandible. Such rare but life‐threatening oral cancers can be correctly diagnosed and treated only through systematic analysis of clinical findings and examining the diseased tissue with histological and other diagnostic procedures. [ABSTRACT FROM AUTHOR]