We describe a Turkish woman with the association of classical cutaneous features of EDS, extensive cerebral calcification and multiple emphysematous bullae. A 20-year-old woman with a 8-year history of EDS with mild chronic lung disease presented with headache. Physical examination revealed fragility and hyperelasticity of the skin and hypermobility of the joints. A chest computed tomography (CT) showed bilateral paraseptal emphysematous areas and bilateral bullous disease. A cranial CT revealed bilateral extensive calcifications in the cerebral and cerebellar hemispheres and basal ganglia with periventricular and subcortical white matter. Cerebral calcification should be carefully searched for using MRI in patients with EDS and neurological symptoms, such as headache.