Rhabdomyosarcoma is a disease that predominantly affects children. Approximately 40 per cent are located in the head and neck region but it is rare in the oral cavity.

This article describes an interesting case of an embryonal rhabdomyosarcoma in a 36-year-old male, involving the mandibular gingiva. The lesion showed radiolucency with ill-defined margins that was crossing the midline. The history revealed a similar lesion six months back at the same site and the lesion had been completely excised. The biopsy reports confirmed the diagnosis of embryonal rhabdomyosarcoma after which en-bloc resection of the tumor was performed with administration of chemotherapy and radiotherapy.

Due to high recurrence rate of rhabdomyosarcomas in adults, multimodal therapy should be planned for proper care of the patient. Clinical, radiological, histopathological and management aspects are discussed here.