Unlike smooth muscle elsewhere in the body, the smooth muscle of the iris and ciliary body is derived from neuroectoderm (mesectoderm). Leiomyomas that arise from the ciliary body, and therefore are of mesectodermal origin, may resemble spindle cell neurogenic tumors by light microscopy. They show positive immunostaining for smooth muscle actin but negative staining for neural markers. Ultrastructurally, the cells have the features of smooth muscle cells. The authors report a typical case of mesectodermal leiomyoma in a 47-year-old woman in which skeinoid fibers, considered to be an ultrastructural marker of neurogenic spindle cell tumors, were frequent together with other ultrastructural features often seen in neuroglial cell tumors. The findings indicate that mesectodermal leiomyoma is unique in its histogenesis as well as in its morphology.