The neurilemmoma is a relatively uncommon benign neoplasm which is known to be originated from the schwann sheath of the nerve fiber. Parapharyngeal neurilemmomas may originate from any nerve traversing this space, but the vast majority arise from the vagus nerve and sympathetic chain. The neurilemmomas arising from the extracranial portion of the hypoglossal nerve are extremely rare. To our knowledge, the case we present is the ninth one to be reported occurring in the parapharyngeal space. Recently we experienced a case of parapharyngeal neurilemmoma arising from the hypoglossal nerve and so we report our case with a brief review of literatures.