Purpose. To evaluate the effect of physiotherapy on ataxia and functional capacity in an individual with Kinsbourne syndrome. Methods. A case study of a 25-year-old male diagnosed with Kinsbourne syndrome undergoing a physiotherapy program in an in-hospital environment for 4 weeks, 5 times a week, with 40-minute sessions. Results. The results in the Scale for the assessment and rating of ataxia (SARA) score decreased significantly after the physiotherapeutic intervention, revealing a significant reduction of ataxia symptoms. The Functional independence Measure (FiM) scores differed between the pre-and post-physiotherapeutic intervention, with improvement in the activities of the self-care domain and transfers. Conclusions. The study showed that after physiotherapy, the patient with Kinsbourne syndrome improved his functional capacity and ataxia pattern, suggesting that a physiotherapy intervention may improve coordination and activities of daily living in people with this rare disease.