Transitional cell carcinoma (TCC) of the ovary is a rare recently recognized subtype of ovarian epithelial cancer. Ovarian TCC has a modest response to chemotherapy, and metastatic TCC from the renal pelvis results in mortality. The clinical presentation is indistinguishable from other types of ovarian carcinoma. Histopathological examination remains the first tool used in the diagnosis of these heterogeneous tumors and in the separation of closely related tumors. Since it is generally accepted that surgical resection is the primary therapeutic approach, and patient outcomes following chemotherapy are better than for other types of ovarian cancers, it is a reasonable concept to detect tumors when they are still confined within the ovaries. Thus, the aim of this review was to describe typical cases of primary TCC, and to review the medical literature for information on TCC management in order to determine appropriate diagnostic methods and therapy.