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Sacrococcygeal Teratoma (SCT) showing pulmonary differentiation has been rarely reported in the literature. Till date, only eight cases have been reported, out of which six belong to teratomas arising from female genital tract. Spinal teratomas showing pulmonary differentiation are rarer and only two cases have been reported so far in the literature both of which had associated congenital spinal anomaly. We present a rare case of seven day old infant with sacrococcygeal teratoma showing mature lung differentiation as one of its components. The infant was otherwise asymptomatic and had no associated spinal anomaly. This case has been presented for its rarity. Extensive review of the literature along with the possible pathogenesis is also included for better understanding.