Background Extrinsic compression of left atrium (LA) due to esophageal achalasia is uncommon. Patients might present with dysphagia, dyspnea, and even hemodynamic compromise. Prompt detection with thorough differential diagnosis is crucial for subsequent management. We presented a case with LA compression by esophageal achalasia, and literature review regarding the clinical manifestation, diagnosis, and treatment strategy was performed to provide an updating knowledge of the disease.Case presentation A 59-year-old relatively healthy man presented with dysphagia accompanied by chest tightness and breathlessness after a large meal. His chest X-ray film disclosed a widened mediastinum. The barium swallow esophagogram revealed contrast pooling at the esophagogastric junction with a bird beak shape. Meanwhile, the transthoracic echocardiogram showed a round-shaped, well bordered, hyperechogenic, and heterogeneous mass (5.1 cm x 3.8 cm in size) compressing the LA irrespective of the systolic or diastolic phase. A chest contrast-enhanced computed tomography scan showed diffuse esophageal dilatation with a smoothly thickening wall aligned compressing the LA. Due to the abovementioned image findings, extrinsic compression of LA by esophageal achalasia was diagnosed.ConclusionLA compression due to esophageal achalasia is not common. Remarkably, given a patient presenting dysphagia and concurrent dyspnea after meals, the clinicians should keep this differential diagnosis in mind. Echocardiography and esophagography are useful to ensure the diagnosis promptly.