Congenital dermal sinus tract is a rare dysraphism with an incidence of 1/2500-3000 ratio. The sinus ostium located in the midline of the posterior of the body from the occiput to the lumbosacral region. Paramedian congenital dermal sinus tract with paramedian ostium are rare. Here, we describe a 2-year-old boy who presented with paramedian pigmented lesion with an ostium on the left lumbosacral region. During surgery we noted that sinus tract attached to thick lipomatous filum terminale.