The clinical features, treatment, and course of 6 patients with multiple myeloma, who presented initially with spinal cord compression, are reviewed. These 6 represent 12% of all patients with myeloma seen at our institution since 1979. Eighty-three similar patients were identified from the medical literature. There are no patient characteristics predictive for this presentation. A higher than expected proportion of patents have clinically localized disease, but progression to myelomatosis is common. As these patients present without a known diagnosis of malignancy, a prompt, invasive diagnostic procedure and the immediate institution of definitive local therapy is mandatory. Despite such an aggressive approach, the prospect for significant return of neurologic function is poor. Survival does not appear to be different from that of other patients with myeloma. Unlike other malignancies, a presentation with cord compression is not inconsistent with long-term survival, although significant morbidity secondary to the resultant functional disability can be expected.