Type I Chiari malformations consist of herniation of the cerebellar tonsils through the foremen magnum and present with a variety of symptoms and signs described previously but hemiathrophy of extremities and hypothalamic hypogonadism are not characteristic findings. Hemiathrophy of extremities has been reported in just one case, and to our knowledge hypothalamic hypogonadism has not been reported previously in literature. In this paper, we reported a unique patient with hemiatrophic upper and lower extremity, short stature and hypothalamic hypogonadism in a patient with type I Chiari malformation