Background  Mycobacterial infections are a serious public health problem worldwide. Involvement of the anal canal and perineum is very rare, but constitute an important differential diagnosis with other equally serious pathologies that may affect the region, such as malignant neoplasms and Crohn’s disease. Objectives  To conduct a literature review on mycobacterial infections of the perianal region considering the most recent information for diagnostic and therapeutic guidance of this disease. Methods  Research was performed on the PUBMED and LILACS databases with the expressions Mycobacterium, Anal, Infection and Tuberculosis. We reviewed articles referring to series of treated cases, clinical reports and literature review published since 2005. Results  Information was compiled on the epidemiology of mycobacterial infections; the clinical behavior of affected individuals; diagnostic options and their validity in clinical practice; and, finally, therapeutic options. Conclusions  Mycobacterial infections of the anus and perineum are rare. The most common clinical presentations are the presence of ulceration and fistulization. The diagnosis involves more than one procedure for identifying the bacilli and should consider the presence of manifestations in more than one organ. The treatment is based on pharmacological intervention. Surgery is recommended for acute complications or chronic sequelae of the disease.

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