A rare case of a 27-year-old male patient with disseminated sacral hydatidosis is presented. Because the diagnosis was missed initially, the patient underwent only partial resection of the tumour to obtain tissue for histology. The resection was followed by deep wound infection, and re-exploration had to be performed, thereby resecting the remaining cyst tissue and the S1-S3 vertebral bodies. Adjuvant anti-helminthic therapy was started postoperatively. Unfortunately, the hydatid cyst further progressed and could only be controlled with multiple decompression procedures and continuance of anti-helminthic therapy. We review the diagnosis, treatment and prognosis of this uncommon condition, which is a serious challenge for the spinal surgeon.

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