Ectopic cilia (EC) are a very rare condition with only few cases reported in literature. Many associations were seen with ectopic cilia which include distichiasis, choristoma and aberrant lacrimal gland, hypochromic nevus, atopic eczema and others. We are reporting a case of an 11-year-old girl with isolated left upper lid ectopic cilia, which was confirmed by surgical removal and histopathological study.