Müllerian cyst of the mesentery and retroperitoneum is an extremely rare disease entity. Only two photomicrograph reports have been published. A 47-year-old Korean woman presented with abdominal distention. A mesenteric cystic mass, measuring 25 × 23 × 18 cm, was found between the liver and right adnexa without any connection with adjacent organs. The cyst was multilocular with gray-white, rubbery walls of relatively uniform 0.2 cm thickness. Microscopically, the lining cells were ciliated and cuboidal to low columnar with an incomplete lining of smooth muscle bundles, adipose tissues, lymphovascular spaces, and lymphocytic aggregates. Ultrastructurally, the lining cells had intercellular junctions, and cilia or microvilli on their luminal surface. The lining cells were immunoreactive for cytokeratin, epithelial membrane antigen and vimentin. To the best of our knowledge, this is the first demonstration of ultrastructural and immunohistochemical findings of Müllerian cyst of the mesentery and retroperitoneum in the English literature.