An extremely rare case of a granular cell tumour arising from the right Kiesselbach's area (Little's area) of the nasal septum is reported. A 69-year-old Japanese woman consulted our clinic and her chief complaints were of continuous serous discharge, stuffiness and occasional slight bleeding from the right nasal cavity. Fibrescopy showed a multilocular mass, which was provisionally considered a nasal polyp. Surgical excision was attempted. During surgery, the tumour shrank markedly following local application of adrenaline, suggestive of hypervascularity. The tumour was successfully excised by careful dissection after cauterisation of the mucosa surrounding the tumour. Histopathological examination revealed morphological features of granular cell tumour immunopositive for S-100 protein. This is the first report of granular cell tumour arising from the Kiesselbach's area in the English literature. The eccentric behaviour of the tumour and the management of a granular cell tumour arising from this area are discussed, together with a literature review.