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Primary cutaneous T-cell lymphomas NOS (CTCL not otherwise specified) involve a heterogeneous group of neoplasms that affect the skin, with no evidence of extracutaneous involvement at the time of diagnosis. The association with hemophagocytic lymphohistiocytosis (HLH) syndrome has been documented. They are rare in children, and usually are characterized by a dismal prognosis. We report an unusual case of a pediatric patient diagnosed as acute pityriasis lichenoide et varioliformis (PLEVA), with a torpid clinical course, association with HLH and a subsequent diagnosis of double negative (CD4/CD8) primary CTCL, with expression of cytotoxic molecules.