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Authors report an extremely uncommon case dorsally exophytic conus dermoid in a three- years old boy, who underwent meningocele repair locate at lumbosacral region at an age of one month. The boy presented with low backache and difficulty in passing urine. Magnetic resonance imaging evaluation of spine revealed presence of a large exophytic mass located dorsally in the conus and the features suggestive of dermoid. He underwent surgical intervention during surgery lesion was dorsally exophytic containing cheesy material with hairs, excision of dermoid along capsule was carried our successfully. Pertinent literature and management of exophytic conus dermoid is discussed briefly.