Blastomycosis is an invasive fungal disease caused by Blastomyces dermatitidis and the recently discovered Blastomyces gilchristii. The medical charts of 64 patients with confirmed cases of blastomycosis in northwestern Ontario during a 10-year period (2004 to 2014) were retrospectively reviewed. The number of patients diagnosed with blastomycosis in Ontario was observed to have increased substantially compared with before 1990, when blastomycosis was removed from the list of reportable diseases. Aboriginals were observed to be disproportionately represented in the patient population. Of the patients whose smoking status was known, 71.4% had a history of smoking. 59.4% of patients had underlying comorbidities and a higher comorbidity rate was observed among Aboriginal patients. The case-fatality rate from direct complications of blastomycosis disease was calculated to be 20.3%; this case-fatality rate is the highest ever to be reported in Canada and more than double that of previously published Canadian studies. The clinical characteristics of 64 patients diagnosed with blastomycosis are summarized.