Osseous cystic echinococcosis (CE) is one of the most complicated and devastating conditions caused by Echinococcus granulosus. Its management is difficult and there is scant literature about it. Areas covered: A literature review was performed to provide an update on its diagnosis, treatment and follow-up. Expert commentary: In most cases diagnosis of osseous CE can only be confirmed by surgery. Osseous CE should be managed by experienced physicians and addressed as a chronic disease with therapies must be aimed at controlling the disease and its sequels or complications, rather than with a curative intent.