Two cases of Polycystic hydatid disease (PH) are reported from the same municipal district of the Brazilian Amazon region (Sena Madureira, Acre). Both had a similar clinical presentation and course over two years of follow-up. Initially the patients complained of pain in the right hypochondrium or upper abdomen and presented obstructive jaundice, fever, increased abdominal volume and weight loss. By image analysis, in addition to splenomegaly, multiple and coalescent cysts were detected in the liver. Serum samples were reactive by counterimmunoelectrophoresis. Treatment with albendazole resulted in partial improvement, with symptomatic relief and reduction in size of the lesions. This report stresses the importance of performing clinical-epidemiological studies of polycystic hydatid disease in the Brazilian Amazon and especially in the municipality of Sena Madureira where many other cases of PH may remain undiagnosed.