Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi. It usually presents as sessile or pedunculated granular polyp, red in colour with multiple yellowish pin-head sized dots. Atypical presentations due to involvement of extra-nasal sites may produce diagnostic dilemma. Very high incidence of rhinosporidiosis and that too with atypical presentations in the rural western part of West Bengal, encourages us to undertake this study. The present study was conducted in a tertiary care hospital in the rural western part of West Bengal from July 2013 to December 2016. Patients presenting with rhinosporidiosis in extra-nasal sites and with atypical presentations were included in the study. Rhinosporidiosis confined to nose and patients who lost follow up were excluded from the study. All patients were treated with wide excision and cauterization of base. Among total 114 patients of rhinosporidiosis, 16 had atypical presentations (14.04%). Nine patients (56.25%) presented with a mass hanging in the oropharynx, some mimicking oropharyngeal malignancy. Two patients (12.50%) presented with acute respiratory distress and stridor. One patient (6.25%) presented with disseminated rhinosporidiosis with involvement of the skin, subcutaneous tissue, muscle, bone, penis and urethra. Recurrence was noted in only two patients (12.50%) in nasopharynx. This chronic disease may present with different acute presentations. Proper clinical eye may avoid pre-operative biopsy which may lead to extensive bleeding. Recurrence can be reduced with meticulous and complete removal. Regular post-operative follow-up with endoscopy is must to detect and treat early recurrence.