Pure primary ovarian squamous cell carcinoma (SCC) is a rare lesion that usually arises from the malignant transformation of an existing ovarian dermoid cyst. The de novo occurrence of an ovarian SCC in the absence of a prior ovarian dermoid cyst, Brenner tumor or endometriosis is extremely rare. At present, no effective therapy exists for treating pure primary ovarian SCC. The present case study describes a patient that presented with progressive coughing, who was diagnosed with an International Federation of Gynecology and Obstetrics stage IV pure primary ovarian SCC with lung metastases. The patient received postoperative chemotherapy, however, the patient succumbed to the disease. The current study also presents a review of the literature.