Intraocular leiomyoma is a rare ophthalmic tumor that is generally believed to be impossible to differentiate clinically from amelanotic uveal malignant melanoma. Based on their personal experience with three cases of intraocular leiomyoma and a review of the literature, the authors describe certain features of leiomyoma that serve to differentiate this tumor from uveal melanoma. The leiomyoma occurs in younger patients and had a distinct predilection for females. It tends to affect the ciliary body and rarely occurs purely in the choroid. The tumor seems to grow in the supraciliary or suprachoroidal space between the uvea and the sclera and it does not seem to arise from the uveal stroma. If the tumor can be recognized clinically the best management seems to be local surgical resection by partial lamellar sclerouvectomy.