Chordomas are rare tumors of the axial skeleton originating from remnants of primitive notochord and occurring in the midline from clivus to sacrum, anterior to the spinal cord. Historically 50% cases were estimated to originate in the sacrococcygeal region, 35% in skull base, and 15% in mobile spine¹. Surveillance, Epidemiology, and End Results (SEER) database analysis of 400 patients from 1973-1995 suggested an almost equal distribution in the skull base (32%), mobile spine (32.8%), and sacrum (29.2%). The incidence is 0.08 per 1,00,000, with the prevalence being twice as high in males compared to females. The peak incidence is seen between 40 to 60 years of age, and proportion of cases in first two decades has been reported as low as 2.6%-5% of all^2-3. No risk factors as such have been identified, however, patients of tuberous sclerosis are at higher risk for the same⁴. These slow-growing malignancies are locally aggressive, but have recurrence rate upto 66% by eight to ten years, depending on the treatment delivered⁵. Distant metastases to lung, bone, soft tissues and liver have been reported in upto 43% patients on prolonged follow-up of 16 years and as low as 5% at presentation or on shorter follow-up duration of five years^6-7. These figures imply that metastases occur mostly late in the course of disease. Multiple studies have attributed the risk of developing distant metastases to local recurrences, with the risk being much lesser in the absence of local recurrences⁸. We present a rare case of rapidly progressive, both locally and distally, case of poorly differentiated sacral chordoma with histologically proven lung metastases at presentation in a child. Being a case report, this article was not required to be reviewed by the ethics committee. Also, informed consent from the patient’s (minor) father has been taken regarding the same.