Occipital Aneurysmal bone cyst is a rare benign tumor-like mass of the children and young adults. It is more frequent to the long bones and to the spine, but its location to the cranium is unusual. It is a lytic lesion, causing bone augmentation with a heterogeneous bloody content. Since its first description in 1942, only few cases have then been published in the literature and all have been listed in the text. We discuss through a successfully managed case of a 2 years old child the pathogenicity, the morphologic and radiologic criteria suggestive of this unusual pseudo-tumor and also discuss treatment options.