A series of 231 consecutive patients with myelomeningocele was reviewed for what concerns the Chiari II malformation. This was present in 228 subjects (98.8%), but was symptomatic in 74 patients (31.5%) and required surgery just in 26 cases (11.4%). First treatment was addressed to hydrocephalus which was always associated. Shunting reverted the symptoms in 12 patients, but failed in 14 who required craniocervical decompression. Mortality consisted of two cases (one early and one delayed). All the remaining patients achieved satisfactory results.