Introduction: Spinal Coccidioidomycosis, although rare, represents the most frequent osseous presentation of Coccidioides spp infection. The present review aims to describe the anatomical distribution, the epidemiological characteristics, and the diagnostic and therapeutic approach of this severe infection. Methods:A meticulous review of all published spinal Coccidioidomycosis cases was carried out. The studied population's demographics and the anatomical distribution of the infection were recorded. Furthermore, the medical and operative management as well as the disease outcome were studied. Results:Seventy-six cases (of which 78.9% males) with a mean age of 35.5 years were located. Regarding the anatomical distribution of the infection, the thoracic area was the most commonly affected spine region (26.3%). Among the studied patients, 14 (18.4%) were immunocompromised. Pain was the most commonly reported symptom (21.1%). Regarding the diagnostic approach of this infection, plain x-ray or CT scan indicated the disease in the majority of cases (44.7%). Pathology (48.7%), serology (42.1%) and microbiological examinations (35.5%) further established the firm diagnosis, with Coccidioides immitis being the most frequently isolated fungus. Medical management included mainly amphotericin B (in 57.9% of cases), followed by fluconazole (in 38.2% of cases). The disease required surgical intervention in the majority of cases (76.3%), while the infection had a successful outcome in 80.3% of cases. Conclusion:Spinal Coccidioidomycosis seems to require prolonged medical treatment, including proper antifungal therapy and, in most cases, operative management. Multidisciplinary approach, including infectious disease specialists, orthopaedic and/or spine surgeons, microbiologists and radiologists seems to be of utmost important for yielding favorable outcomes.