Neurilemmomas are slow growing, benign neoplasms of neural crest Schwann cell origin. They arise from any peripheral, spinal or cranial nerve except the olfactory and optic. Presentation is usually asymptomatic but focal neurological signs and symptoms may be associated with nerve compression. With approximately one third of all documented cases presenting in the head and neck region, we report a case of a submandibular neurilemmoma misdiagnosed pre-operatively. The diagnostic difficulties are discussed and the current literature reviewed. Thiscase highlights the importance of inclusion of nerve sheath tumours in differential diagnoses of soft tissue lesions in the head and neck.