This is a neuropathological report of the youngest case ever recorded of tectocerebellar dysraphia with posterior encephalocele. All previously described stigmata were ascertained as well as a few additional findings. Some morphological features of this syndrome similar to and some different from the Dandy-Walker syndrome and the Cleland-Chiari (Arnold-Chiari) anomaly are briefly discussed. Finally the possibility of clinical diagnosis and favourable prognosis of this syndrome are suggested.