Arnold Chiari malformation is a common congenital anomaly of the craniocervical junction. It is defined as a descent of the cerebellar tonsils below the foramen magnum, putting pressure on both the brain and spine causing many symptoms. The patients typically experience no symptoms or chronic slowly progressive symptoms. However, recent reports indicate that a subset of patients with AC may present with acute deterioration, cardiorespiratory arrest and sudden unexpected death. We report a case of sudden unexpected death of a 34 years old woman, married with 3 kids, without any medical history, that was admitted for a heterogeneous multinodular goiter in clinical and biological euthyroidism and scheduled for total thyroidectomy. In intraoperative, the patient presented a cardiorespiratory arrest while positioning her head in hyperextension for a good surgical approach, neck released by anesthesia. Para-clinical tests showed later an AC malformation. Cases of sudden cardiorespiratory arrest in children and adults with AC malformation have been documented, leading to death in some cases. However, these patients who died unexpectedly have experienced a recent trauma. This is a unique example of a sudden unexpected death of a woman undiagnosed AC, in absence of any previous trauma, that occurred during a conventional anesthesia for a thyroid surgery.