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A 19-yr-old woman with a previous history of a mass of the right ciliary body presented with a decreased visual acuity of right eye. Clinicoradiologic examinations suggested a recurrent mass of the ciliary body. Enucleation of the right eye was performed under the impression of malignant tumor. On microscopic examination, the tumor was a mesectodermal leiomyoma of the ciliary body. On immunohistochemistry, the tumor cells were reactive to smooth muscle actin and vimentin, but not reactive to cytokeratin, S-100 protein, neurofilament, desmin, epithelial membrane antigen, HMB-45, glial fibrillary acidic protein, and synaptophysin. Electron microscopy revealed numerous thin longitudinally placed myofilaments and focal densities in the cytoplasms. In the review of the literature, only 27 cases of mesectodermal leiomyoma of the ciliary body were reported, however, there was no report of recurrent cases. Mesectodermal leiomyoma should be differentiated from other orbital spindle-cell tumors such as amelanotic melanomas and glial tumors. Immunohistochemical and electron microscopic studies may be useful for the correct diagnosis by showing smooth muscle differentiation in the tumor cells
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