Pericardial effusions leading to cardiac tamponade have previously been described with esophageal cancer. However, up to eighty percent of these cases have been reported in association with chemotherapy and radiation. Patients with esophageal cancer seldom initially present with pericardial effusion resulting from esophageal pericardial fistula (EPF). Herein, we present the case of a 62-year-old man who presented with pericardial effusion with an unknown etiology at presentation. Subsequently, the patient developed cardiac tamponade and was referred to the tertiary hospital for further evaluation. Computed tomography of the chest revealed a circumferential irregular enhancing lesion at the mid-thoracic esophagus suspecting esophageal cancer with EPF and a moderate amount of pericardial effusion. The patient underwent esophagoscopy and squamous cell carcinoma was found from the esophageal biopsy. An esophageal stent was successfully placed to conceal the perforation. Eventually, the patient died 13 days after admission complicated by refractory septic shock. This case highlights an atypical presentation of esophageal cancer and an unusual cause of cardiac tamponade.