Ruptured esophageal varices can present as sudden death from gastrointestinal hemorrhage. The most common underlying pathology causing esophageal varices is cirrhosis leading to portal hypertension. However, not all esophageal varices arise from portal hypertension, and not all portal hypertensions are caused by cirrhosis. We present a rare case of ruptured esophageal varices casing death in an individual with metastatic tumor (high-grade) neuroendocrine tumor in the liver causing portal hypertension. This is, to the best of our knowledge, the first case report in the literature reporting a neuroendocrine tumor causing esophageal varices. This case report aims to document this rather rare entity, highlight another mechanism on how metastatic disease can result in sudden death, and give a brief review of literature on metastatic tumor in the liver causing esophageal varices.