Progressive Supranuclear Palsy (PSP) is a rare degenerative disease showing supranuclear ophthalmoplegia, rseudobulbar palsy and several parkinsonian features. This is , as far as the author's knowledge, the first case in this country. The patient is a 55 year old female with slowly progressive disturbances in walking, swallowing, speech and behaviors. The most characteristic finding is the prominent external ophthalmoplegia, especially vertical and downward gaze, which can be easily overcome by the oculocephalic reflexes (Doll's eye movements). The dyspagia, dysarthria and emotional lability may suggest the pseudobulbar palsy. General hyper-reflexia and Babinski sign are noted. There are several features of parkinsonism, but the rigidity is more severe in the trunk than in the extremities and the posture is rather hyperextended than scooped. There is no tremor. The disease is slowly progressive and the medical treatment for Parkinson's disease is not effective.