A newborn infant had a craniopharyngioma. Review of three similar cases from the literature together with a description of our case is the subject of the present paper. Distinct presenting features are hydrocephalus, huge suprasellar calcification, and high protein ventricular fluid level. Ventriculoatrial shunt is the procedure of choice to palliate the hydrocephalus. We also feel that surgical approach to the tumor should be limited to biopsy and decompression during the neonatal period.