: Neuroendocrine tumors rarely occur in the cervix or other components of the reproductive system. These tumors have been associated with microsatellite instability, are very aggressive and often associated with poor outcome. Lynch syndrome is an inherited cancer syndrome that has also been associated with microsatellite instability. Here we report a 34-year-old female with Lynch syndrome and a family history of loss of DNA mismatch of the hereditary non-polyposis colorectal cancer repair gene expression who presented with a neuroendocrine tumor of her cervix as the first manifestation of Lynch syndrome. This is the first case reported of a neuroendocrine tumor of the cervix in a patient with Lynch syndrome. We also review the relationship between Lynch Syndrome and neuroendocrine tumors.