Background: Primary ovarian non Hodgkin’s lymphoma (PONHL) is a very rare disease. We present a case of PONHL and review the literature. Case: The patient, a 24-year-old nulliparous Greek woman, presented with the complaint of abdominal pain. She underwent left salpingo-oophorectomy, multiple biopsies from the right ovary, total omentectomy, pelvic and paraortic lymphadenectomy, appendectomy and curettage. The histopathology revealed diffuse large B-cell non-Hodgkin’s lymphoma of the left ovary. She underwent postoperative chemotherapy. She remains well without evidence of disease, 15 months after initial surgery. Conclusion: The use of chemotherapy is based on the principle that PONHL must be considered a localized manifestation of systemic disease. Patients with PONHL have a similar outcome compared to patients with other NHL.