PurposeThe aim of this paper is to discuss the problems of craniocervical instability and craniocervical fusion in infancy. Despite the relative frequency of carniovertebral joint malformations, actual instability is quite rare in infancy.MethodsAn infant 8 months of age presented with tetraparesis and sleep apnea due to a complex malformation of her craniovertebral joint. An initial attempt at conservative treatment using a rigid neck collar failed, so the patient was surgically managed by the onlay placement of two autologous rib grafts. The rigid collar was maintained for 5 months.ResultsBoth rib grafts progressively reabsorbed within a few months, while the clinical deficits recurred. Reoperation consisted of occipitocervical interposition of two robust struts of banked cadaveric adult fibula. This time, the skull appeared …