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Echinococcus granulosus remains a clinical problem in sheep and subsistence farming communities in South Africa. The most commonly affected organs are the liver and the lung. Most cysts remain clinically silent and are diagnosed incidentally or when complications occur. Clinical examination is unreliable in making the diagnosis. Serological testing has a broad range of sensitivity and specificity and is dependent on the purity of the antigens utilised. Ultrasound examination of the abdomen is gens utilised. Ultrasound examination of the abdomen is both sensitive and cost effective. Computed tomography and endoscopic retrograde cholangiopancreatography (ERCP) are reserved for complicated cases. The differential diagnosis includes any cystic lesion of the liver. Liver hydatid cysts can be treated by medical or minimally invasive (laparoscopic and percutaneous) means or by conventional open surgery. The most effective chemotherapeutic agents against the parasite are the benzimidazole carbamates, albendazole and mebendazole. Albendazole is more efficacious, but recommended treatment regimens differ widely in terms of timing, length of treatment and dose. Medical treatment alone is not an effective and durable treatment option. PAIR (puncture, aspiration, injection, reaspiration) is the newest and most widely practised minimally invasive technique with encouraging results, but it requires considerable expertise. Open surgery remains the most accessible and widely practised method of treatment in South Africa. The options are either radical (pericystectomy and hepatic resection) or conservative (deroofing and management of the residual cavity). Various scolicidal agents are used intraoperatively (Eusol, hypertonic saline and others), although none have been tested in a formal randomised controlled trial. Laparoscopic surgery trials are small and unconvincing at present and should be limited to centres with expertise. Complicated cysts (intrabiliary rupture and secondary infection) may require ERCP to obtain biliary clearance before surgery, and referral to a specialist centre may be indicated.