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Abstract This paper aims to introduce a patient with Chiari type 1 malformation presented with upper extremity pain and diabetes insipidus. After laboratory examinations, we confirmed our case's central diabetes insipidus diagnosis. The patient has undergone posterior fossa decompression, tonsilopexy, and duraplasty. After 6 months of follow‐up, pain and diabetes insipidus were improved. We introduced a rare presentation of Chiari malformation in this article.