Strongyloides stercoralis (SS) is endemic in tropical and subtropical areas worldwide and in the southeastern United States. The lifecycle of SS is both unique and complex. Human infection begins with the penetration of skin by filariform larvae that migrate hematogenously to the lungs. Larvae then ascend the airway, are swallowed, and mature in the gut. Unlike other nematodes, SS can autoinfect the same host and persist for decades. Categorization of infection includes acute, chronic-uncomplicated, and disseminated forms. Clinical manifestations depend on the particular organs involved. Fifteen to thirty percent of chronically infected people may be asymptomatic. On the other hand, SS may cause the adult respiratory distress syndrome, septic shock, and death. The diagnosis of SS infection is suspected in patients from endemic areas who have blood eosinophilia, and gastrointestinal or pulmonary symptoms. A definitive diagnosis is established by demonstration of SS larvae in stool, body fluids, or tissues. A presumptive diagnosis of SS infection can be achieved by serology. Thiabendazole is the mainstay of treatment, but repeat doses may be necessary if the parasite is not initially eradicated. The low incidence of disseminated SS in areas endemic for both SS and AIDS is surprising and unexplained.