The simultaneous presentation of bifid nose and bilateral cleft hand defects has not previously been described. Already rare in presentation, the bifid nose continues to challenge reconstructive surgeons as to optimal management. Although cleft defects of the hand may be somewhat more common, the multitude of surgical approaches available to correct these deformities is a testament to their lack of optimal effect. From an etiologic standpoint, it would seem that facial and hand clefting might share little in common. However, our recent experience with managing these coincident deformities in a newborn prompted a re-evaluation of material related to this exceedingly rare event. Here, we present a 4-month-old female infant with a coincident bifid nose and central ray deficiency.