Alveolar echinococcosis caused by Echinococcus multilocularis is an infrequent zoonosis with a high degree of disability, morbidity, and mortality, especially in disease clusters of the northern hemisphere. The diagnosis is complicated by extended incubation time, diverse clinical manifestations, and mimicking of differential diagnoses. The primary organ affected is the liver, but extrahepatic disease is possible, with vertebral involvement in only a few dozen cases described worldwide. Although vertebral alveolar echinococcosis seems to be rare, it might be under diagnosed, and it might be seen more often as the number of people with immunocompromised conditions increases. Recognition of this syndrome is crucial, because advances in medical and surgical management strategies since the introduction of benzimidazole in 1976 have controlled and relieved symptoms in most cases. In this Grand Round, we present the case of a 75-year-old woman who was referred for biopsy of a lumbar lesion 3 months after she was diagnosed with chronic myeloid leukaemia. The diagnosis of hepatic alveolar echinococcosis with metastasis to the lumbar spine and paravertebral region as well as the brain was confirmed by biopsy, PCR, and serology. The patient was given albendazole and referred for palliative surgery with the aim of pain control. Clinical features of the case are presented and discussed in the context of the literature. This case and review illustrate the complexity of extrahepatic alveolar echinococcosis manifestations and the necessity of an interdisciplinary approach.