Primary small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is an extremely rare neoplasm of the neuroendocrine tumor (NET) family, diagnosed mainly in young women in their second and third decade. In 50-75% of the cases it coexists with mostly asymptomatic hypercalcemia. Due to highly non-specific and cryptic course, much like ovarian cancer, in about half of the cases it is detected in far-advanced clinical stages. Determination of final diagnosis is possible only after surgery, based on comprehensive microscopic and immunohistochemical studies. Management of a primary SCCOHT diagnosed in a 19-year-old woman is discussed in detail. Initial surgical treatment did not result in a correct histological diagnosis. The patient underwent combined treatment, including surgery and chemotherapy. Over 3 years’ follow-up, she has been operated on six-fold. Even using several protocols of adjuvant chemotherapy after surgery, consecutive recurrences of the disease could not be prevented. To date, no uniform management protocol has been developed, due mainly to great rarity of this tumor type and its extremely aggressive behavior. Advanced cases require aggressive surgical treatment, including hysterectomy with adnexectomy and omentectomy. Considerable clinical dilemma is posed by young women, who wish to preserve fertility. Some authors suggest that faced with such an aggressive tumor type, even in early-stage cases, preservation of fertility in young women should not be taken into account. In late-stage cases, cytoreduction is recommended. Adjuvant and neoadjuvant chemotherapy of the pulmonary type (cisplatin + etoposide) is an important part of the therapy. In view of very aggressive behavior of the tumor, selected cases may require high-dose chemotherapy with autologous stem cell rescue (HD-SCR) as first-line adjuvant treatment or, even more frequently, as consolidation treatment. In early-stage cases, great hope is associated with the use of adjuvant radiotherapy after surgery and chemotherapy. SCCOHT is a rare condition with an aggressive clinical course and poor prognosis, where treatment outcomes are still far from satisfactory. In spite of treatment, most patients die during the first two years. Inability to perform large-scale randomized trials results in lack of optimal and universally accepted management protocol. Close cooperation of highly specialized oncologic centers is necessary to promote our understanding of biology of this tumor type.