Sex cord tumor with annular tubules (SCTAT) is a rare ovarian pathology. It is usually an incidental finding in asymptomatic patients. In about 30% of cases, this tumor is associated with the Peutz-Jeghers syndrome (PJS). This case report describes an incidental finding of SCTAT during an excisional surgery of a giant mucinous cystadenoma in the contralateral ovary. In addition to the finding of a rare tumor in association with a rare syndrome, a literature review on the diseases, their association, and management was performed.
