Small Cell Carcinoma of the Ovary, Hypercalcemic Type -SCCOHT is a rare and extremely malignant gynecological tumor. Both diagnosis and treatment are currently a complex problem in clinical practice, and the prognosis of patients, despite their young age at diagnosis, remains consistently unfavorable. The aim of the study is to present current knowledge about SCCOHT, as well as the results of preclinical studies constituting potentially new therapeutic strategies, based on available literature in the Pub Med database. Promising results have been achieved by research on epigenetic therapies, CDK4/CDK6 inhibitors, immunotherapy, tyrosine kinase inhibitors, as well as the elimination of arginine from the tumor environment. However, individual case reports and the results of preclinical studies available in the PubMed database indicate that in order to improve the long-term results of SCCOHT treatment, combination therapies have to be considered.