Summary

Parry–Romberg syndrome (PRS) or progressive hemi facial atrophy syndrome is a rare condition of unknown aetiology that is characterised by progressive unilateral facial and cranial atrophic changes of skin, subcutaneous tissues and bone. The authors describe a 37-year-old female with a history of PRS, who presented with a subarachnoid haemorrhage secondary to rupture of a 9 mm fusiform aneurysm of the posterior cerebral artery. There was an associated external carotid arterio-venous fistula noted with this aneurysm. The aneurysm was treated by endovascular route and was successfully coiled. Follow-up angiogram revealed spontaneous resolution of the fistula with good occlusion of the aneurysm. The aetio-pathogenesis of this rare occurrence, literature review and its management is discussed.