Aneurysmal bone cysts of the sphenoid sinus are very rare, with only six ca
ses described in the literature. We present a case of an aneurysmal bone cy
st of the sphenoid sinus with associated fibrous dysplasia in which the rad
iological findings had some features of a mucocoele. We discuss the differe
nces in pathogenesis, clinical presentation and radiological appearances be
tween these two lesions, and propose a simple drainage procedure as an effe
ctive modality of treatment.