Esophageal cancer is an aggressive malignancy associated with high morbidity and mortality. Due in part to a lack of effective screening programs, esophageal cancer is usually at least locally advanced at the time of diagnosis. The two major histologic subtypes (adenocarcinoma and squamous cell carcinoma) are associated with a high rate of local advancement and metastasis at the time of diagnosis, which occurs by lymphangitic and hematogenous spread. A biopsy is required to confirm the diagnosis, determine histologic subtype, and obtain tumor markers to identify potential therapy options. Treatment is based on the clinical stage of tumor and patient risk factors dictating suitability for surgery and systemic therapy. While esophagectomy is one of the mainstays of treatment for resectable tumors, it is commonly performed after neoadjuvant treatment, except in early-stage cancers limited to the mucosa. Surgical resection involves removal of the entire esophagus or lower two-thirds, followed by reconstruction with an appropriate conduit, such as the stomach or colon. After surgery, patients with residual tumor are offered adjuvant immunotherapy for 1 year. Patients are followed closely with surveillance cross-sectional imaging to detect disease recurrence.