RESUMO
OBJECTIVE: To present an extremely rare case of sebaceous carcinoma arising in a mature cystic teratoma of the ovary. CLINICAL PRESENTATION AND INTERVENTION: A 66-year-old woman presented with abdominal discomfort and a pelvic mass. Abdominal and pelvic ultrasound, as well as CT scan, revealed a 27 cm complex right pelvic mass, which was diagnosed histologically as a sebaceous carcinoma arising in a mature cystic teratoma. The patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy, peritoneal washings, appendicectomy and infracolic omentectomy. CONCLUSION: This case adds to the rare reports in the literature of sebaceous carcinoma occurring in a mature cystic teratoma. The clinical behaviour and optimal management of this entity are not well established. The patient has been well for 32 months following surgery with no evidence of recurrent disease clinically.