Cholangiocarcinoma most commonly presents as painless progressive jaundice. We report a case occurring in a 56-year-old Chinese woman with an unusual presentation of progressive dysphagia and vomiting. Oesophageal manometric and barium studies were indicative of achalasia, and computed tomography confirmed the presence of cholangiocarcinoma extending to the gastroesophageal junction and proximal lesser curve of the stomach. In this case, a constricting tumour at the gastroesophageal junction with probable invasion of the vagus nerves led to features of achalasia and gastroparesis.