A 48 year old woman with no significant prior medical history was found dead by her husband in their home. The autopsy disclosed no anatomic reason for her death; however, the length of the esophagus was found to be massively dilated, with stenosis of the cardiac sphincter, and contained swallowed food material. Her husband disclosed that she had experienced difficulty in swallowing for over 10 years, and had to "strain" to move food into the stomach, although she had never consulted a physician regarding the problem. No gross or microscopic anatomic cardiac abnormalities were identified. The death was ascribed to a cardiac arrhythmia arising from the Valsalva maneuver, which she used to move her ingested food across the stenotic gastroesophageal juncture. The Valsalva maneuver, which increases the intrathoracic pressure by forcing expiratory effort against a closed glottis, has been associated with cardiac arrhythmias and rarely, sudden death. Lethal cardiac arrhythmias should be considered when sudden deaths occur in individuals with esophageal motility disorders, as well as in other situations where the Valsalva maneuver may have been used, and where no other anatomic cause of death is identified.