Since malignant transformation in a dermoid cyst is extremely rare, the clinicopathological characteristics of patients with squamous carcinoma arising in dermoid cyst are evaluated in the hope of offering a more rational therapy. Our first patient was lost to follow-up after surgery. In the second patient, with a gross stage III disease, after extensive surgery and multiagent chemotherapy we were able to achieve a remission period of 42 months. In the third patient with pelvic lymph node metastases, adjuvant radiation and chemotherapy was administered following surgical staging. She was free of disease 6 months after surgery. Malignant transformation in a dermoid cyst is a rare complication observed especially in older age groups. Thus, a frozen section in these decades may improve detection of this complication allowing a more accurate staging. Although poor prognosis is reported, an aggressive approach with cisplatinum based chemotherapy and radiation and even with secondary cytoreduction, long-term remission may be achieved.