Objective To study the cl inical characteristics of sacrum echinococcosis disease so as to provide a basis for its diagnosis and treatment. Methods Between July 1999 and August 2010, 14 cases of sacrum echinococcosis disease were treated and the cl inical data were analysed retrospectively. There were 6 males and 8 females with an average age of 28.7 years (range, 16-58 years). The median disease duration was 5.7 years (range, 6 months to 11 years). Twelve cases came from cattle area or had a history of close contact with dogs and sheep; 12 patients had a history of lung or l iver hydatid disease. The main clinical symptoms were lumbosacral and lower extremity pain, one or both lower extremities and perineal hypoesthesia, the gradual dysfunction of both lower extremities and neurogenic bladder and bowel, and decreased force of urination. The imaging examination showed multicystic bone erosion. The Casoni test was performed in 10 cases and the results were positive in 8 cases. The eight immunoserology markers of hydatidosis were detected in 4 cases, and the results were all positive. After entire debridement of the focus, defects were repaired with autogenous bone, allogeneic bone, artificial bone, or bone cement, and then albendazole was used as a routine treatment for 3 months. Results Incisions healed by first intention, and no compl ication occurred. All patients were followed up 6 months to 11 years with a median time of 5.7 years. The preoperative cl inical symptoms were rel ieved. Eight cases recurred at 6 months to 3 years after operation; after debridement (3 times in 5 and 5 times in 1) and the lesions marginal resection with a high-speed burr (2 cases), the symptoms were relieved in different degrees without recurrence. No hydatid disease occurred and spreaded. Conclusion The cl inical manifestation of sacrum is not typical, which is easy to be misdiagnosed. MRI is helpful to diagnosis sacrum echinococcosis disease; serological examination is the major method of identification diagnosis, and surgery is the main treatment method.