Total absence of ribs is a rare finding that has occasionally been documented in patients with cerebrocostomandibular syndrome. Only 2 other reports document complete absence of ribs in 3 individuals, and we tabulate the findings of all the 4 cases of complete absence of ribs in this case report. Our case and the other 3 reported cases represent the extreme form of cerebrocostomandibular syndrome with regard to the costal defects.