Syringomyelia is a disorder in which a cyst or cavity forms within the spinal cord. This cyst, called syrinx, can expand and elongate over time, destroying the spinal cord. We describe the case of a young patient with partial spontaneous regression of syringomyelia in Chiari I malformation, confirmed by magnetic resonance imaging three years after the diagnosis. During this period the patient did not experience any clinical symptoms. Although described in literature, spontaneous regression is an unusual event and very few cases have been reported. This case report supports the belief that conservative management together with both clinical and imaging periodic controls should be preferred in stable mild-symptomatic patients.