Posterior scleritis in an 18-year-old female with primary familial brain calcification syndrome: Incidental finding or association?

Primary familial brain calcification (PFBC) is an idiopathic neurological disease associated with intracranial calcification. An 18-year-old female, a known case of uncontrolled Type 1 diabetes mellitus, presented with unilateral progressive diminution of vision in her left eye. Based on her clinical examination, a diagnosis of left eye posterior scleritis was made. Computed tomography of the brain revealed bilateral caudate and lentiform nuclei patchy calcifications suggestive of Fahr's syndrome or PFBC. In our patient, age and posterior scleritis were two uncommon presentations to be associated with PFBC.