Small cell carcinoma of the ovary is a rare and often lethal tumor, occurring primarily in young women. In as many as two-thirds of reported cases there is an associated paraneoplastic hypercalcemia, the symptoms of which are often what bring the patient to medical attention. This phenomenon also provides a convenient biochemical marker useful for follow-up. The origin of these tumors is unknown. Recent studies using flow cytometry would appear to distinguish these tumors from other ovarian tumors based on their DNA diploid histograms. These tumors have a very poor prognosis, and a proven regimen of adjuvant chemotherapy is yet to be defined. A case of a young woman with small cell carcinoma of the ovary with hypercalcemia, alive, with no evidence of recurrence 5 years after surgery followed by chemotherapy is reported.