Primary malignant lymphoma involving the ovaries is extremely rare. We present a unique case of a primary Non-Hodgkin's lymphoma (NHL) of both ovaries, preceded by an internal jugular vein trombosis (IJVT) as paraneoplastic syndrome. Currently, 36 months after surgical treatment of this FIGO Stage Ib, Ann Arbor Stage 2E NHL, the patient is clinically free of disease. Based on this case and a review of the literature it is concluded that paraneoplastic syndromes like spontaneous IJVT should prompt the clinician to make a thorough diagnostic work-up in search of an underlying malignancy, including the female genital tract.