<p class="abstract">Extracardiac rhabdomyoma is a very rare condition with potentially multifocal manifestations including the head and neck region. We present a case of an 81-year-old patient who presented as a medical emergency because of ambiguous, progressive throat pain and dysphagia. After diagnostic work up including magnetic resonance (MR) scan and biopsy a rhabdomyoma of the tongue base was diagnosed. The tumor was completely excised by transoral laser microsurgery and the patient’s symptoms vanished. This case report is described in context of the current literature with a discussion of diagnostic steps and treatment options of rhabdomyoma in the head and neck region. Rhabdomyoma is a rare, but nevertheless relevant differential diagnosis of head and neck tumors.</p>