Scopulariopsis is a common fungus in the environment, characterized by its intrinsic resistance to the available antifungal drugs. Around 70 cases of infection by this fungus have been described in the literature. Pulmonary and disseminated infections are the most common and their treatment is difficult; therefore, very diverse approaches have been taken, with varied results. A successful outcome has been reported in only a few cases, generally attributed to a multitreatment strategy combining medical and surgical procedures that ultimately led to the resection of the infected tissue if possible, identification of the mould, and an aggressive long-term antifungal therapy. Although most of the infections are caused by Scopulariopsis brevicaulis, a few other species have also been linked to these cases, although molecular evidence has not been proven for all of them. On this basis, more knowledge on the epidemiology, presentation, diagnosis, treatment, and prognosis of these unusual infections would improve their management. This review aims to compile the current data on Scopulariopsis infections.