The medullary conus represents a distinct entity of the spinal cord regarding its anatomical, clinical and microsurgical features. An overview of the pathologic processes of this region is provided. Epidemiological, clinical and neuroradiological characteristics of neoplastic (glial tumors, non-glial tumors, metastasis, primary melanomas) and non-neoplastic lesions (granulomatous lesions, abscess, parasitic infections, vascular, demyelinating and dysembryogenetic lesions) are discussed. Main MR imaging characteristics used to differentiate neoplastic from non-neoplastic lesions consist in pathological spinal cord expansion, gadolinium-enhancement and tumoural cyst formation. Management strategies differ substantially, depending on the kind of lesion. According to the suspected pathological entity radical resection, biopsy or conservative treatments are reasonable options. Intraoperative electrophysiological monitoring is a fundamental part of the surgical setting.