Katayama syndrome is an early clinical manifestation of schistosomiasis that occurs several weeks post-infection with Schistosoma spp (trematode) worms. Because of this temporal delay and its non-specific presentation, it is the form of schistosomiasis most likely to be misdiagnosed by travel medicine physicians and infectious disease specialists in non-endemic countries. Katayama syndrome appears between 14–84 days after non-immune individuals are exposed to first schistosome infection or heavy reinfection. Disease onset appears to be related to migrating schistosomula and egg deposition with individuals typically presenting with nocturnal fever, cough, myalgia, headache, and abdominal tenderness. Serum antibodies and schistosome egg excretion often substantiate infection if detected. Diffuse pulmonary infiltrates are found radiologically, and almost all cases have eosinophilia and a history of water contact 14–84 days before presentation of clinical symptoms; patients respond well to regimens of praziquantel with and without steroids. Artemisinin treatment given early after exposure may decrease the risk of the syndrome.