The isolated unilateral palsy of the hypoglossal nerve is an unusual sign in daily medical practice. We present the case of a 74 year old man, without relevant medical history, who started with headache and difficulty to swallow. On the physical examination, he had isolated hypoglossal nerve palsy without evidence of tongue atrophy or fasciculation. The patient had a good spontaneous evolution. Idiopathic isolated hypoglossal nerve palsy requires an exhaustive evaluation with neuroimages and complementary exams. It usually presents partial or total spontaneous remission.