Background: Achalasia is a condition characterized by the absence of peristaltic in the lower esophageal body and hypertonic of the lower esophageal sphincter (LES), caused by degeneration of the myenteric plexus ganglia, leading to inadequate relaxation during swallowing. Case Presentation: A 33-year-old woman complained of dysphagia when consuming solid or liquid food since last year. The patient also reported heartburn and significant weight loss. Chest X-ray finding was unremarkable. However, endoscopic examination revealed a narrowed esophagus with food residue and a rosette appearance. Contrast-enhanced abdominal computed tomography (CT) scan showed luminal obstruction at the level of CV Th12, resulting in dilation of the proximal esophagus and food retention. After failure of oral medication therapy, definitive therapy in the form of heller myotomy was performed. The patient showed significant improvement after the procedure with good oral intake tolerance. Discussion: The exact etiology of achalasia remains unknown. The gold standard for diagnosing achalasia is manometry, but this modality is not available. Diagnosis of achalasia in this patient started with clinical symptoms of dysphagia, heartburn, and weight loss, supported by endoscopic and contrast-enhanced abdominal CT scan findings. Other management options include pneumatic dilatation and peroral endoscopic myotomy (POEM), but heller myotomy remains the recommended management with a higher success rate. Conclusion: Achalasia is a rare motor disorder of the esophagus and the exact etiology of achalasia remains unknown. The diagnosis of achalasia was concluded from clinical symptoms, physical examination, and supportive investigations (endoscopic examination and contrast-enhanced abdominal computed tomography). Management of achalasia in this patient consisted of administering oral medication and definitive therapy in the form of heller myotomy with good results.