This general review of the biomedical literature indexed on Medline (PubMed) and Pascal (INIST) over the past 40 years (1970-2010), supplemented by some unindexed studies, found 89 articles published about basidiobolomycosis. These case reports (n = 67) and series (n = 22) described 172 cases of basidiobolomycosis (84 in Asia, 47 in tropical Africa, 25 in North America, 12 in South America, two in Europe and two in Australia). Patients younger than 15 tears accounted for 70%, and the sex-ratio (M/F) was 2.1. Clinically, basidiobolomycosis results in firm subcutaneous plaques, sharply circumscribed, generally cold and painless, becoming hot and painful during flares. It can cause invasive disease of the gastrointestinal tract or lungs and can even be disseminated throughout the body. The main treatments are potassium iodide, trimethoprim-sulfamethoxazole, and the azole derivatives. The latter are very effective and well tolerated, unlike the former, which present a risk of recurrence or severe side effects.