Introduction: Granulomatous inflammation tries to isolate and control a foreign substance considered hard to eliminate. Intramedullary granulomas are rare, but some can be deadly. Objective: To point out the initial reports, differential diagnosis and prognosis of these lesions. Material and Methods: A systematic review related to granulomatous spinal lesions was performed in the PubMed, MedLine (EBSCO), LILACS and Scielo database using the keywords “granulomatous”, “granuloma” and “intramedullary”. Results: intramedullary granulomatous lesions most commonly are present in the thoracic spine (80% of cases). The most frequent cause of the lesion was tuberculosis, followed by sarcoidosis. Improvement of the symptoms was found in 78%, almost two thirds of this total. Most cases were treated with surgery and medication with 47% of total improvement of symptoms. Sixty seven percent of patients treated only with medications were fully recovered. None of the four patients that were submitted only to surgery fully recovered. Conclusion: Given the most frequent causes are tuberculosis and sarcoidosis, it is suggested to always investigate them. Although the combined treatment was the most used, the drug alone showed greater outcomes. However, more studies are needed to make it possible to confirm the efficacy of those treatments.