The prevalence of cocaine use is rising worldwide, with a resultant rise in associated pathology. Regular nasal use can cause cocaine-induced midline destructive lesions (CIMDL), which can be difficult to distinguish from ear, nose, and throat (ENT)-limited Wegener's granulomatosis (WG). Two cocaine users presented with mid-facial pain, epistaxis, and systemic symptoms. Both had nasal septal perforation, necrosis of sinus mucosa, and positive anti-neutrophil cytoplasmic antibodies (ANCA). Histology was inconclusive and treatment with immunosuppressive drugs was commenced. The first patient continued to use cocaine initially, with improvement in her symptoms only on high doses of steroid. Later she stopped cocaine and this plus a switch from cyclophosphamide to mycophenolate mofetil resulted in successful symptom resolution and steroid withdrawal. The second patient denied cocaine use but having only partially responded to high-dose prednisolone and methotrexate, she admitted continued cocaine use and was lost to follow-up. Evaluation of a patient with destructive lesions of the mid-face should include enquiry about intranasal use of cocaine. Localized ENT involvement, inconsistent ANCA pattern, and atypical biopsy findings for WG should be recognized as features of CIMDL. Although cessation of cocaine use is crucial, there may be a role for immunosuppression.