We report a rare clinical association of optic disc melanocytoma (ODM) occurring with bilateral congenital ptosis that has not been previously reported. A 28-year-old male patient presented with bilateral congenital ptosis. On examination, his vision was 20/20 in each eye, and dilated fundus examination indicated a pigmented lesion over the left inferior temporal aspect of the optic disc that was consistent with an optic nerve melanocytoma. B-scan ultrasonography indicated a calcified elevated optic nerve head. Bilateral frontalis sling procedures were performed for congenital ptosis with satisfactory results at 1 year postoperatively. There was no progression of the lesion in the left eye. Knowing the key ophthalmoscopic features of an ODM can aid in diagnosing this lesion; ophthalmologists should be familiar with this lesion in addition to the rare association of ptosis. Affected patients should be periodically assessed.