Human herpesvirus‐8 (HHV‐8) infection is associated with neoplastic and non‐neoplastic diseases in immunocompromised patients. Kaposi sarcoma (KS) is a common malignancy reported in solid organ transplant recipients (SOTR). Kaposi sarcoma inflammatory cytokine syndrome (KICS), initially described in HIV patients, is characterized by high viral loads, elevated levels of cytokines, cytopenia, high fever, organ failure, and poor outcome. We report the case of a 54‐year‐old patient who developed simultaneous occurrence of KS of lymph nodes and KICS as a complication of primary donor‐transmitted HHV‐8 infection, after heart transplantation (HT). The diagnosis, management, and prognosis of this condition are unclear and needs a multidisciplinary approach.

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