Summary: An 18‐year‐old female Fjord horse presented with a history of recent progressive dysphagia, coughing and increased respiratory effort during exercise, which had not improved with administration of systemic antimicrobials. Upper airway endoscopy, radiography and computed tomography showed a soft tissue mass at the dorsal aspect of the larynx in the region of the proximal cervical oesophagus. Humane euthanasia was opted for and post‐mortem, histological and immunohistochemical examination provided a definitive diagnosis of leiomyoma of the proximal oesophagus. Neoplasia, and more specifically a smooth muscle neoplasm, of the proximal cervical oesophagus has not previously been reported in horses. [ABSTRACT FROM AUTHOR]