The history, clinical signs and pathological findings in seven adult horses with histologically confirmed idiopathic granulomatous disease, primarily of the lungs, are reviewed. They ranged in age from eight to 21 years, five were geldings and two were females, they belonged to five breeds and there were no seasonal or geographical associations. The primary clinical signs were chronic weight loss, exercise intolerance and respiratory distress which did not respond to conventional treatment. The most consistent physical findings were depression, anorexia, tachycardia, tachypnoea and adventitious lung sounds. Thoracic radiographs revealed a diffuse, structured, nodular, interstitial pulmonary pattern in each horse. Haematological measurements suggested a chronic inflammatory process and the cytology of transtracheal washes was consistent with a mild suppurative inflammation. Idiopathic granulomatous pneumonia was confirmed histologically in each of the horses, either postmortem or by a lung biopsy. The horses responded poorly to medical treatment and only one of three treated horses is still alive.