Background  Mucocutaneous leishmaniasis is a granulomatous disease clinically characterized by ulcerated skin and mucosal lesions. Mucocutaneous leishmaniasis is very rare in India and to our knowledge, only two cases have been reported, and this is the first case of mucocutaneous leishmaniasis presenting with mutilating rhinopathy reported from the Indian subcontinent.

Case report  A 64-year-old man presented with a destructive ulceration of the central face of 23 years’ duration, who was diagnosed to have mucocutaneous leishmaniasis, and showed dramatic response to intramuscular injections of sodium stibogluconate.

Results  Histopathologic examination of skin biopsy revealed a granulomatous infiltrate with the presence of leishmania donovani (LD) bodies. The clinical picture, plus the pathologic findings, and the response to sodium stibogluconate confirmed mucocutaneous leishmaniasis.

Conclusion  Mucocutaneous leishmaniasis is a rare disease in the Indian subcontinent, and clinicians from this region should have a high index of suspicion on encountering mucocutaneous ulcerative lesions.