We herein describe a rare case of ulcerative colitis associated with unilateral hypoglossal nerve palsy. A 64-year-old woman developed severe active ulcerative colitis and was treated with prednisolone. The dose of oral prednisolone was reduced to 7.5 mg/day, following which the patient noticed slight dysphagia and a speech disturbance. She was diagnosed with unilateral hypoglossal nerve palsy, which was thought to be caused by mononeuritis. She was treated with intravenous methylprednisolone at a dose of 500 mg/day, which improved the neuropathy. When the neuropathy occurred, the patient was in a mildly active stage of ulcerative colitis. We concluded that the mononeuritis observed in the present case was likely an extraintestinal manifestation of ulcerative colitis.