Puff of smoke appearance on cerebral angiography is seen in:

Moyamoya disease is an idiopathic, non inflammatory, non atherosclerotic progressive vasculo-occlusive disease involving the circle of Willis, typically the supraclinoid internal carotid aeries. Small abnormal net-like vessels proliferate giving the characteristic "puff of smoke" appearance on direct angiography. CTA and MRA is not always able to demonstrate this appearance on account of lower flow and spatial resolution. REF : DAVID SUTTON 7th ed