Ossifying fibromyxoid tumour is a recently described, rare but morphologically distinctive soft tissue neoplasm characterised by a combination of myxoid and/or fibrous stroma with areas of ossification. Although most authors postulate a neuroectodermal origin for this peculiar tumour, there is no agreement in the literature regarding its histopathogenesis. To our knowledge, this is the first reported case of ossifying fibromyxoid tumour involving the sphenoid sinus.

Tumour of low cell density, composed of small, spindle-shaped or stellate cells with small, irregular nuclei set in a fibromyxoid stroma.

Following discussion at the skull base multidisciplinary team meeting, a combined surgical team including an otorhinolaryngologist and a neurosurgeon carried out resection of the lesion, using an endoscopic transnasal approach, followed by reconstruction of the defect.

An awareness of the distinctive histopathological features of ossifying fibromyxoid tumour, and of its clinical effects, is crucial to establishing a definitive diagnosis and thereby instituting appropriate management. This case report also reinforces the evolving role of the endoscopic transnasal approach in the management of inflammatory and neoplastic disease involving the skull base. This is increasingly being made possible by close collaboration between multiple surgical specialties, including otorhinolaryngology and neurosurgery.