Michelin Tire Baby Syndrome is a rare congenital disorder and characterized clinically well defined multiple ring shaped skin creases. Our patient was born to non-consanguineous healthy parents as the third child of the family at 40 weeks of uneventful gestation with distinctive skin creases and gigantism. He was 4,950 g in weight (> 90 percentile), 57.5 cm in length (> 90 percentile), and had a head circumferences of 39.5 cm (> 90 percentile) at birth. The physical examination showed a rough face, brachicephaly and craniosynostosis. His vital and laboratory findings were within normal limits at birth. Cranial and renal ultrasonograms, X-ray graphics and cytogenetic analyses were normal. Echocardiography revealed small patent ductus arteriosis and patent foramen ovale. In this report, we present a new case of Michelin Tire Baby Syndrome who is the first neonate associated with severe gigantism and craniosynostosis, in the literature. A review of the related literature has also been presented.