A 26-year-old male patient from India, resident in Italy since 2005, presented in March 2006 to the Department of Otorhinolaryngology with a history of nasal obstruction of three months duration. The family history of the patient revealed a similar disease in his mother, which recurred twice (in 1994 and in 2000). The patient referred recurrent epistaxis since the age of 7, treated with cold packs, and a not better specified conjunctival inflammation cured with eyedrops in September 2005. Physical examination showed an erythematous, papillomatous mass, 3 cm in diameter, obstructing the right nasal cavity, attached by a narrow pedicle to the nasal septum. No abnormality was seen in the controlateral nasal cavity or nasopharynx. The mass was resected endoscopically and the base of implant was electrocoagulated. The patient did not assume any drug therapy and, until today, after five months of follow-up, during which he underwent a clinical exam twice, he is healthy with no sign of recurrence. The excised mass weighed 4 grams, measured a maximum diameter of 3 cm, was pink, with a fleshy consistence, studded with whitish spots on its surface. On histological examination, the lesion showed the characteristic features of the rhinosporidiosis: the polypoid fibroconnective stroma, covered by flat multi-stratified squamous epithelium, contained many globular cysts. Each of these cysts represented a thick-walled sporangium containing numerous "daughter spores" in different stages of development. The stroma contained a vascular fibroconnective tissue with fibroblasts and myofibroblasts and an inflammatory infiltrate (neutrophil granulocytes, lymphocytes, plasma cells and histiocytes). Histochemical stains such as PAS, GMS and mucicarmine were used to establish the correct diagnosis of rhinosporidiosis. Morphological criteria were based on the diameter of the endospores and sporangia, respectively 5–10 μm and 50–1000 μm. These findings made easier the distinction of Rhinosporidium seeberi from another common nasal mycosis aetiological agent, Coccidioides immitis.