A review of seven cases of intraocular leiomyoma personally managed by the authors disclosed clinical and histopathologic characteristics that serve to differentiate this uncommon tumor from uveal melanoma. Leiomyoma generally occurs in younger patients and has a definite predilection for females. It tends to affect the ciliary body and peripheral choroid rather than the posterior choroid. In contrast to melanoma, which is located in the uveal stroma, leiomyoma usually is located in the supraciliary or suprachoroidal space. During transillumination, leiomyoma usually transmits light readily, whereas most melanomas cast a shadow. If intraocular leiomyoma is suspected clinically, the best management seems to be removal by a modified lamellar sclerouvectomy. In contrast to melanoma, leiomyoma shows positive immunoreactivity for muscle markers and negative immunoreactivity for melanoma-specific antigen and neural markers.