We present the case of a 3‐year‐old female liver transplant recipient with a history of Caroli disease who presented with positive severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) reverse transcription polymerase chain reaction (RT‐PCR) test and was ultimately diagnosed with multisystem inflammatory syndrome in children (MIS‐C) complicated by portal vein thrombosis. To the best of our knowledge, this is the first case report of MIS‐C in a pediatric solid organ transplant (SOT) recipient. Based on our patient, MIS‐C could be a potential complication of Coronavirus disease 2019 (COVID‐19) in SOT recipients and may have a negative outcome on transplant graft function.

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