Abstract: Objective　To investigate the clinical features and prognosis of Kaposi varicelliform eruption with severe complications. Methods　The clinical data of one child with Kaposi varicelliform eruption with severe complications was retrospectively analyzed. The related literatures were reviewed. Results　A 5-month-old boy presented with recurrent rash on the head and face for 3 months and aggravated for 3 days. The skin lesions showed a characteristic of typical dome-shaped blisters with hemorrhagic crusting. At admission, the boy suffered with severe hypoproteinemia, hypocalcemia, and electrolyte disorder. The hypocalcemia was aggravated gradually. On the fifth day of admission, the boy had fever, convulsions, and tachycardia. Blood culture showed methicillin-resistant Staphylococcus aureus (MASA) infection. The diagnosis of sepsis was confirmed. At that very day, the boy started to have coagulopathy, so Fusidic and Vancomycin for anti-infection, Acyclovir for antivirus, intravenous infusion immunoglobulin, albumin, cryoprecipitate, plasma and calcium gluconate were administered, supplied with albumin and blood coagulation factor. The boy’s condition gradually became stable and discharged on the 19th day after admission. Conclusions　When Kaposi varicelliform eruption is complicated with hypoproteinemia and hypocalcemia, the critical illness is indicated. Clinicians should be alerted to the existence of sepsis, coagulation disorders, even septic shock and disseminated intravascular coagulation.