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Sweet's Syndrome also knows as acute febrile neutrophilic dermatosis, is a rare skin's condition, that can occur either idiopathic or secondary. In the case of the latter, the syndrome can develop after certain malignancies (paraneoplastic syndrome), because of exposure to some medication or post infectious. It is more frequent in women aged between 30 and 50 years, but concerning children, the disorder is extremely rare (8% of the total number of cases), having equal sex ratio distribution. We present the case of an 11 year old male, diagnosed with systemic form of SS associated with Myelodysplastic Syndrome. The onset of the hematological condition seemingly occurred at the age of 5, when the diagnosis of chronic immune thrombocytopenic purpura was established. The treatment included repeated cortisone administrations, followed by a splenectomy procedure. Admitted in our Oncopaediatric department in December 2012, the child is given the diagnosis of MS, to which severe systemical manifestations of SS were added, with partial treatment response (cortisone, cyclosporine, dapsone, indomethacin). An allogeneic bone marrow transplant was conducted at Fundeni Institute (February 2015) when the SS remission occurred, but the progression was fatal, the child developing graft-versus-host disease. [ABSTRACT FROM AUTHOR]