We report two cases of combined hamartoma of the sensory retina and retinal pigment epithelium (CHR-RPE) in which apparent growth of the lesion was observed. In case 1, the eye was enucleated with a presumed diagnosis of juxtapapillary malignant melanoma. Histopathologically, the enucleated globe showed an elevated peripapillary mass containing disorganized retinal tissue intermixed with vascular and glial elements as well as tubules of proliferating retinal pigment epithelium. We have summarized the clinical features of 53 patients with CHR-RPE reported between 1952 and 1988 excluding the cases compiled by the Macular Society Collaborative Study. While the latter study found an equal sex predilection among their cases, we found a 70% male preponderance among the 53 patients. Of the 54 lesions observed in 53 patients, 76% were juxtapapillary, 17% were macular, and 7% were peripheral. Furthermore, periodic follow-up examination disclosed apparent enlargement of the mass in six patients, five of whom underwent enucleation of the globe for suspected melanoma.