OBJECT: In this paper the authors describe an association between increased body mass index (BMI) and Chiari malformation Type I (CM-I) in adults, as well as its relationship to the development of syringomyelia. METHODS: In the period between January 2004 and December 2011, the senior author reviewed the data for all CM-I patients with or without syringomyelia and neurological deficit. Analyzed factors included clinical status (headaches and neurological signs), radiological characteristics of syringomyelia (diameter and vertical extent of syrinx), BMI, and relationship of age to BMI, syrinx diameter, and vertical extent of syrinx. RESULTS: Sixty consecutive adults had CM-I, 26 of whom also had syringomyelia. The mean BMI among all patients was 30.35 ± 7.65, which is Class I obesity (WHO), and was similar among patients with or without syringomyelia. Extension of the vertical syrinx was greater in overweight patients (p = 0.027) than in those with a normal body weight. Evidence of de novo syrinx formation was found in 2 patients who gained an average BMI of 10.8 points. After repeated decompression and no change in holocord syrinx width or vertical extent, a reduction in the syrinx was seen after BMI decreased 11.7 points in one individual. No correlation was found between patient age and BMI, age and vertical extension of the syrinx, and age and diameter of the syrinx. CONCLUSIONS: An association between increased BMI and CM-I in adults was recognized. Gaining weight may influence the de novo creation of a syrinx in adults who previously had minimally symptomatic or asymptomatic CM-I, and reducing weight can improve a syrinx after unsuccessful surgical decompression. Therefore, a reduction in body weight should be recommended for all overweight and obese patients with CM-I.