OBJECTIVE To highlight the characteristics of giant cell interstitial pneumonia (GIP). METHODS The clinical, radiological and pathological data of a patient with GIP confirmed by open lung biopsy were presented, and relevant literatures were reviewed. RESULTS Patients with GIP usually had a history of exposure to metal dust. Clinical presentations include cough and dyspnea on exertion, and pulmonary function testing showed a restrictive abnormality. On chest radiography and high-resolution CT scans, it presents as bilateral areas of ground-glass attenuation, areas of consolidation, diffuse small nodules, extensive reticular opacities and traction bronchiectasis. The main pathological findings include a desquamative interstitial pneumonia (DIP)-like reaction with intra-alveolar macrophages and numerous large multinucleated histiocytes that ingested inflammatory cells were admixed with macrophages. The finding of GIP is almost pathognomonic for hard metal pneumoconiosis. CONCLUSIONS GIP is a very rare chronic interstitial pneumonia, and has no characteristic clinical manifestations. Radiographic findings are similar to other idiopathic interstitial pneumonias. Meticulous history taking on occupational exposure is important for the diagnosis of GIP.