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Transitional cel carcinoma (TCC) of the ovary is a rare and recently recognized subtype of ovarian epithelial cancer. We presented the first case report from our Institute (Sheri Kashmir Instiute of Medical Sciences, Srinagar, India), which was initial y misdiagnosed as stromal cel carcinoma (granulosa cel tumour), and on review of histopathology with immunohistochemistry, the diagnosis of TCC of the ovary was established. The aim of this article was to describe the typical case of primary TCC of the ovary and to review the literature for information on TCC management.