Posterior choroidal leiomyoma is a sporadic, rare benign tumor that is always confused with anaplastic melanoma. Here we report a case and provide a review. Most of the preoperative findings in our case were suggestive of malignant choroidal melanoma. However, the contrast enhanced ultrasound (CEUS) suggested a benign hemangioma. In summary, the posterior choroidal leiomyomas were yellowish-white in color and most commonly located in the temporal quadrant of the fundus (11/15). They were more frequent in Asians (13/16), the prevalence was almost equal in males and females (9:7), with a mean age of 35y. Microscopically, the tumor typically showed spindle cell bundles and nonmitotic ovoid nuclei arranged in intersecting fascicles. Vitrectomy is now a popular treatment option and definitive diagnosis can be made after immunohistochemistry. Finally, some summarized features of this tumor differ from those previously described. These may help in the diagnosis of posterior choroidal leiomyoma and differentiation from malignant melanoma.