Chronic granulomatous disease (CGD) is a group of hereditary disorders of impaired intracellular destruction of phagocytosed bacteria. Gastrointestinal manifestations, are present, with hepatic abscess being the most common. In this case report, we present an adult with CGD with esophageal involvement, which has been described in only one other adult. The clinical history, modalities of diagnosis (including endoscopy, barium radiography, and esophageal manometry), and therapeutic strategies pertaining to the esophageal manifestations of CGD are discussed. A review of the pertinent available literature is provided.