Review of the literature concerning cocaine induced midline destructive lesions (CIMDL). We reviewed the English literature regarding CIMDL involving the nose and its surrounding structures. The review is based on a search of the US National Library of Medicine (PubMed) online database from January 1st, 1982 to March 31st, 2013. CIMDL is a pathology that mimics systemic diseases with positive anti-neutrophil cytoplasmic antibodies (ANCA). The prevalence of CIMDL is considered to be about 4.8% among cocaine users. Clinical manifestations include hyposmia, facial pain, crusting, ulcers, nasal septal perforation, palatal perforation, sinus wall destruction, orbital erosion and damage of the anterior skull base. The presence of ANCA directed against human neutrophil elastase (HNE) is the most distinguishing feature of CIMDL. Toxicological tests, indirect immunofluorescence microscopy, antigen specific solid assay testing, histopathological analysis, apoptosis assay and MRI imaging concur in the clinical identification of CIMDL. The pathogenesis of CIMDL is poorly understood and implicates inflammatory, infective, proapoptotic and autoimmune mechanisms. CIMDL must be readily recognized by clinicians to provide appropriate treatment. Immunosuppressive therapy has no role in the treatment of CIMDL. Only abstinence can interrupt the progression of the disease.