<p>Giant cell tumour (GCT) is a benign tumour that originates from undifferentiated mesenchymal cells of the bone marrow. The cranium as well as temporal bone is a rare location for GCTs. Uncommon in paediatric age group. Commonly involved sites are distal femur, proximal tibia, distal radius and sacrum. Malignancy in giant cell tumour is rare. 3% of the tumours may become malignant which has poor prognosis. Surgery is the only effective treatment for the GCTs of the temporal bone. Broad excision is desirable to lower the recurrence rate, but with GCT originating from the temporal bone, it is often difficult to perform complete and radical extirpation. Despite the locally aggressive, highly recurrent nature, GCTs are benign tumors with a good prognosis after wide radical resection. A middle aged male in forties came to OPD with complaints of headache and vertigo and ear discharge since 2 months. There was history of diminished hearing for low intensity sounds. Fragile mass occupying the medial half of external auditory meatus and diagnosis of middle ear tumour was made. HRCT temporal bone showed an intra medullary osteolytic lesion in left temporal bone involving skull base. Erosion of glenoid fossa, left temporomandibular joint, roof of left external auditory canal, anterior wall of left tympanic cavity and tegmen tympani was also noted. Focal extension of lesion into left mesotympanum and posterior part of left EAC was noted. Findings were suggestive of giant cell tumour or plasmacytoma. Patient underwent temporal bone resection with enucleation of tumour under GA and the defect was reconstructed using abdominal fat. Multinucleated cells were osteoclast-like and were associated with clusters of mononucleated cells or lying freely. The features were suggestive of giant cell tumour. The contour of his temporal bone area was maintained due to aesthetic repair. Tumors that can’t be removed surgically can often be controlled and sometimes destroyed with radiation therapy. Giant cell tumors can recur. The role of adjuvant radiotherapy in treatment is still controversial. Radiotherapy is recommended for tumors that are not amenable to complete resection, cases with morbidity limiting the use of general anesthesia, and cases with recurrent progressing lesions despite multiple surgeries. Radiotherapy is used as an adjunct therapy and has been found to be safe and effective in modest doses. Some authors report that radiotherapy may trigger sarcomatous transformation of GCT. A close association between clinical, radiological and histopathological features is essential to make a definitive diagnosis of a rare tumour like Giant cell tumour. Complete resection followed by filling the defect with abdominal fat gives a good aesthetic look maintaining the contour of the temporal bone.</p>