The aim of the study was to describe the clinical and radiological features of childhood post-varicella cerebral infarction (PVCI). A retrospective review was undertaken of children with arterial ischaemic stroke (AIS) who had experienced varicella zoster virus (VZV) infection within the preceding year. Twenty-four children (15 males, nine females; age range at time of VZV infection 2mo-6y) were identified, with a median of 4 months between VZV and AIS (range 1wk-12mo). All had infarction in the middle cerebral artery (MCA) territory and abnormalities of the M1 segment; arteriopathy affected other arteries in 10 children. After a median of 27 months, six patients had recurrent transient ischaemic attacks (TIA), with new infarcts in two of 22 children on re-imaging. Arterial disease improved in 11 children, was stable in four, and progressed in seven (of whom four had recurrent TIA and two had re-infarction). PVCI affects young, previously healthy children within a few months of VZV infection and is characterized by MCA territory infarction and proximal MCA disease. One quarter of patients have recurrence, usually, but not inevitably, associated with progressive arteriopathy. Treatable co-existing AIS risk factors should always be excluded. A more comprehensive diagnostic evaluation should be considered in children with AIS who do not fit the clinical and radiological profile outlined, even where there is a history of recent VZV infection.