Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT), first described by Dickersin et al. in 1982, is a rare and highly aggressive neoplasm that predominantly affects women between the ages of 10 and 40. It accounts for less than 1% of all ovarian cancers, making it an uncommon and challenging entity.

Case Report: A 25-year-old woman presented with suprapubic pain for 3 days, worsening in intensity, and progressive abdominal distension over the past 2 months. On physical examination, she exhibited tenderness on palpation of the lower abdomen and a palpable hypogastric mass. Vaginal examination revealed a tender cervix upon mobilization. Imaging demonstrated a large, heterogeneous mass with ascites. Exploratory laparotomy was performed with resection of a solid left ovarian mass along with the adherent ipsilateral fallopian tube, total hysterectomy, right salpingo- oophorectomy, omentectomy, appendectomy, bilateral pelvic and retroperitoneal lymphadenectomy. Histopathology revealed a high-grade carcinoma, and immunohistochemistry confirmed SCCOHT. Chemotherapy cycles were initiated, but within three months the patient developed malignant bowel obstruction, ascites, and peritoneal carcinomatosis. Due to extensive disease and lack of therapeutic response, she died four months after diagnosis.

Discussion: This disease is typically unilateral, rapidly progressive, and life-threatening. Its histogenesis remains unproven. Pathologic diagnosis is not straightforward due to morphologic overlap with other tumor types. Clinical presentation is variable, often nonspecific, and may include hypercalcemia-related symptoms (up to 90% of cases), though some patients are asymptomatic. Treatment usually involves a multidisciplinary approach with radical surgery, chemotherapy, radiotherapy, and palliative care.