Between 1976 and 1984, 11 patients with stab wounds at the craniocervical junction were seen in the Department of Neurosurgery at Groote Schuur Hospital. The injury usually occurred in males, and the left side was predominantly involved. Because of the anatomical features of this region, the penetrating instrument is deflected by the occipital squama into the atlantooccipital or atlantoaxial interspace, and an almost predictable syndrome occurs. The dura mater is penetrated, so that cerebrospinal fluid leakage and meningitis are common complications (meningitis occurred in 5 patients). A meningocele may develop at this site and did occur in 4 patients, but only 2 required surgical repair. Because of the exposed position of the vertebral artery at this level, this vessel was injured in 4 patients; an arteriovenous fistula developed in 2, vertebral artery occlusion occurred in 1, and a false aneurysm developed in another patient. The neurological deficit varied in magnitude, was often transient, affected the upper limbs more than the lower, was asymmetrical (suggestive of lateralized injury), and at times showed a remarkable tendency to recover. Awareness of the existence of this syndrome may help in forestalling complications. The only warning sign may be an insignificant wound in the suboccipital or retromastoid region.