Recent studies on the epidemiology and control of Guatemalan onchocerciasis, chiefly made by the Guatemala-Japan Cooperative Project on Onchocerciasis Research and Control, are reviewed. Epidemiological features of Guatemalan onchocerciasis are summarized as to characteristic altitudinal distribution of endemic areas, disease manifestation, vector taxonomy, biology and transmission dynamic of the disease. Extensive insecticide studies in the field and laboratory demonstrate that the characteristic situations of Guatemalan streams where Simulium ochraceum, the main vector of onchocerciasis, breeds require ingenious methods of larviciding. Finally, the feasibility of an area vector control is indicated by the successful control operation in the San Vicente Pacaya Pilot Area, in which a new fixed-dose larviciding method was applied.