We report two children with autoinflammatory syndromes treated with anakinra who came in contact with the varicella-zoster virus after being exposed accidentally to infected children: both cases were managed prophylactically with specific antichickenpox intravenous immunoglobulins and anakinra temporary suspension; neither adverse events nor complications related to the natural course of chickenpox were experienced by the two patients. The risk of developing infectious events should be closely monitored, because of the absence of data concerning long-term safety of biological agents in the pediatric age, and prevention strategies should be highly encouraged before they are started