Idiopathic muscular hypertrophy of the oesophagus (IMHO) is reported in equids as an incidental post mortem finding, infrequently associated with clinical signs of oesophageal dysfunction or additional oesophageal abnormalities. Clinicopathological and post mortem findings are presented from a 15-year-old gelding that developed a spontaneous perforation of the distal, intrathoracic oesophagus, in association with IMHO. The majority of oesophageal perforations documented in the equine literature have been related to external or iatrogenic trauma resulting in rupture of the cervical portion of the oesophagus. In this case, the early presenting features included pyrexia and inappetance, and progressed over a 4 day period to include signs of tachypnoea, tachycardia and endotoxaemia due to the development of a fatal septic pleuritis and pleural effusion. Oesophageal obstruction was not a presenting feature. Diagnostic investigation in cases of unexplained septic pleural effusion should include oesophageal endoscopic evaluation to rule out perforation of the intrathoracic oesophagus. IMHO may represent a clinically significant, primary disease entity with the potential to lead to oesophageal perforation.