A 24 year old man came with bone pains and gradually increasing size of the head - mainly in the parietal regions - since six months.
The CT scan of the temporal bones shows extensive sclerotic bony expansion with a ground glass appearance involving both the temporal bones, sphenoid and petrous temporal bone, left parietal bone. The axis is also affected to a lesser extent. There is almost complete bony stenosis of the left external auditory meatus down to 1–2 mm.