Purpose: Congenital spinal dermal sinus tract is a rare lesion connecting skin to deeper structures including neural tissue. It results from the failure of the neuroectoderm to separate from the cutaneous ectoderm in the third to fifth week of gestation. The common locations are the lumbosacral and occipital regions. Sometimes it extends to spinal canal. In this paper we report a case of congenital spinal dermal sinus tract in the coccyx. Methods: A 21-month-old male child born after an uncomplicated full-term pregnancy was admitted to our institute with a midline dermal sinus and a cartilaginous protrusion in the coccygeal region. There were no signs of infection. Neurologic examination showed no functional deficit in both lower limbs. He was treated with complete excision of the tract and an underlying accessory cartilage. Results: The spinal dermal sinus tract was extended from the skin to the coccyx. The stalk was loosely attached to the accessory cartilage of coccyx. At that point, it was dissected from the accessory cartilage and resected. The accessory cartilage was also resected at the bone and cartilage junction. During the follow-up period of 6 months, the wound healed well without any complication nor recurrence. Conclusion: Congenital spinal dermal sinus tract is known as a form of spinal dysraphism. In order to prevent complications, timely surgical intervention including complete resection of sinus tract with correction of associated abnormalities is of utmost importance.