Familial hypercholesterolemia presenting as intracranial xanthoma

A 22 years female who was diagnosed as having cholestseatoma of right ear was referred to us for medical fitness. On examination she incidentally had evidence of tuberous and tendon xanthomas. She was found to have hypercholesterolemia. On mastoid exploration a yellowish groomous mass was seen which was surrounded by foamy macrophages, suggestive of 'mastoid xanthoma'. The purpose of this case presentation is to report occurrence of such rare case and importance of early detection that will warrant treatment with proper diet and medical management. This will stabilize lesions and delay complications.