•Ovarian dysgerminoma associated with paraneoplastic fever, cytopenia and splenomegaly•Complete symptom resolution resulted from tumor resection and medical management•Non-hematolymphoid neoplasms are part of differential diagnosis in secondary HLH.
Keywords: Adnexal mass; Cytopenia; Hemophagocytic lymphohistiocytosis; Splenomegaly; Unexplained fever.