Echinococcosis or hydatid disease affecting the spine is an uncommon manifestation of Echinococcus granulosus infection of the spine. More commonly found in endemic areas, it causes significant morbidity and mortality as it grows slowly and produces symptoms mainly by compressing the spinal cord. As diagnostic methods are non-specific, diagnosis and management are usually delayed until the disease is advanced, thereby therapy is usually unlikely. Treatment is usually surgical, aiming at cyst excision, spinal cord decompression and spinal stabilization. This article summarizes the clinical findings of echinococcosis of the spine, discusses the specific laboratory and diagnostic findings, lists the current treatment options, and reviews the patients’ outcomes. The aim is to prompt clinicians to be aware of the possibility of echinococcosis as a possible diagnosis in endemic areas.Cite this article: EFORT Open Rev 2021;6:288-296. DOI: 10.1302/2058-5241.6.200130