The occipital encephalocele is a rare homozygous congenital malformation, linked to the C677T gene according to Molloy et al [1,2]. In relation to a defect in the closure of the cranial part of the neural tube during intrauterine life, it is therefore defined such as a hernia of the nervous tissue (part of the cerebellum) and / or the meninges through a bone defect in the occipital region. Its incidence is 0.8 to 3.0 per 10,000 births (sex ratio of 0 girls to 9 boys) according to some studies. It can be meningocele (only meninges), encephalocele (only brain tissue) or meningoencephalocele (brain tissue and meninges). Etiologically, several factors have been implicated in their occurrence. According to Lemire and Robert L, these factors act for some malformations during neurilation, i.e., between the first 30 days of gestation and the closure of the posterior neuropore, and for others in the post-neurilation period. Young maternal age and multiparity are also associated with neural tube defects. Clinically, it varies in size ranging from a small tumefaction or even a large painless mole mass covered with skin tissue [1-3].