Congenital oesophageal stenosis is rarely described in horses. This case report describes a neonatal colt with signs of a milky nasal discharge and cervical swelling present from birth. The diagnostic evaluation revealed a triple oesophageal stricture, identified as congenital fibromuscular oesophageal stenosis. A megaoesophagus was seen cranial to the strictures, but its aetiology remains unexplained. To the authors’ knowledge, multiple oesophageal stenosis together with megaoesophagus has not previously been described in the horse.