Extranodal natural killer/T cell lymphoma (ENKL), nasal type, a rare disease presenting with vague non-specific symptoms, can impose great diagnostic difficulties and may masquerade several infectious, rheumatological or neoplastic conditions. Here, the authors report a case of ENKL presenting as a palatal perforation, naso-oral fistula and fever in a 21-year-old man, which was initially misdiagnosed as bacterial osteomyelitis, invasive fungal infection and Wegener's granulomatosis. The case report underlines the possibility of ENKL occurring at a young age, its rapidly progressive and locally destructive nature, associated diagnostic challenges and the importance of immunophenotyping in establishing the diagnosis.