In the endemic countries, human cystic echinococcosis (CE) poses a serious medical and social problem. Because it most often affects the liver and lungs we aimed to define the proportion of cases with different organ localization, the diagnostic and therapeutic approaches in such cases, and the outcome of them. For a period 2010–2019, a total of 2863 cases of CE were registered in Bulgaria, of which 148 (5.17%) with organ localization other than liver and/or lung. The majority of patients with extra-hepatopulmonary localization of CE were adults. The distribution by gender showed predominance of female patients (57.43%) over those of males and primary cases (85.14%) exceeded the cases of recurrence. According to our study most common is the spleen involvement, followed by involvement of the abdominal cavity, kidneys and muscle/subcutaneous tissue. Other extra-hepatopulmonary organ localizations are significantly less common. This study shows that the extra-hepatopulmonary localization of CE is not so rare, and in most cases it is a primary disease. In respect of this, clinicians should consider hydatidosis in the differential diagnosis when cystic formation(s) is found, regardless of the organ involved.