A 31-year-old male from the southern part of India with no known comorbidities presented with painful oral erosions over his palate following a dental procedure. Over 2 months, it progressed to the ...desquamation of blackish necrotic skin, gingival hypertrophy associated with intermittent fever and odynophagia. He had elevated acute-phase reactants. Biopsy of the gingiva showed necrotising vasculitis. ANCA was weakly positive for the cytoplasmic pattern by immunofluorescence. The HRCT chest showed a single cavitating nodule in the right upper lobe and a non-cavitary nodule in the left upper lobe with right maxillary sinus thickening. With the above clinical and biopsy findings, granulomatosis with polyangiitis was the first consideration. He was referred to our centre since the oral lesions did not improve and nodular swellings developed on the palate. The examination showed cervical lymphadenopathy, which was subjected to an excision biopsy. It showed extensive areas of necrosis with karyorrhectic debris and numerous intracellular and extracellular round-to-ovoid spore-like structures. Special stains were positive for histoplasmosis. Abdomen imaging showed adrenal enlargement. Cultures were negative, which could be due to the fact that he was on multiple medications. He was treated with amphotericin and itraconazole. His symptoms improved. But later, he had worsening clinical parameters suggestive of a progressive disseminated form of histoplasmosis. In spite of aggressive efforts, he couldn’t be resuscitated owing to the dissemination of the organism. Even though considered to be more common in immunosuppressed individuals in nonendemic areas, it is reported in immunocompetent individuals as well and should be strongly suspected if the clinical picture fits so for early diagnosis and aggressive management to prevent fatal complications. There have been only fewer case reports of histoplasmosis from the southern part of India. ANCA is only rarely reported false positively in histoplasmosis.