Congenital synkinetic movement of the upper eyelid is rare. In this case report, we present a 4-year-old patient with a left upper eyelid ptosis that improved on abduction and became worse on adduction. The patient also had congenital esotropia and superior rectus palsy. Strabismus surgery was performed, and excellent surgical results were achieved. A 17-mm left levator aponeurectomy and myectomy was later performed with satisfactory results. Pathology studies of the levator muscle and aponeurosis showed no evidence of inflammation, degeneration, or atrophy. Electron microscopy showed no skeletal muscle abnormality. We postulate that this congenital synkinetic movement reflects an incomplete nerve cell dying process that occurs during the embryonic phase of development.