Study Design. A case of an intramedullary spinal cord abscess is presented. The literature on this subject is carefully reviewed. Objectives. To give an overview of clinical presentation, radiographic examination, pathogenesis, treatment, and outcome of intramedullary spinal cord abscesses, Cases presented in the literature are arranged and the findings summarized. Summary of Background Data. Intramedullary spinal cord abscesses are rare. Most frequently, single cases were reported, followed by a short discussion. All previously reported cases were reviewed. Methods. After an extensive literature search, all cases of an intramedullary spinal cord abscess (totaling 93 patients), including ours, were reviewed. Incidence, clinical presentation, neuroradiologic investigations, treatment, and pathogenesis are discussed. Results. Intramedullary spinal cord absesses are rare. The presentation can be very confusing, mimicking thoracic or abdominal diseases. Magnetic resonance imaging is the best diagnostic technique, although the findings are not pathognomonic. Appropiate antibiotic therapy after surgical drainage is warranted. If left untreated, the outcome is bleak. Whereas most cultures remain sterile (38.7%), Staphylococcus or Streptococcus species are involved frequently (23.7% and 17.2%), Conclusions. Although intramedullary spinal cord abscess is a rare disease, every spine surgeon should have knowledge of its existence because misjudgment and deferring adequate treatment may lead to an unfavorable outcome.