Alveolar echinococcosis (AE) is a rare zoonosis caused by the parasite Echinococcus multilocularis. Nothing is known about the health-related quality of life (HRQoL) in patients with AE receiving different types of therapy. Therefore, the aim of the study was to compare HRQoL in patients with AE in Germany depending on their therapeutic regimen namely conservative drug treatment with long-term benzimidazoles versus surgical therapy by resection of the parasitic liver lesions. The 36-Item Short Form Health Survey (SF-36) questionnaire, including other echinococcosis-related questions, was used to measure HRQoL. The SF-36 scales were evaluated according to the algorithms provided by the authors. The statistical analysis was performed with SAS version 9.2. The significance level was set at α = 0.05, p < 0.05 corresponds to statistical significance. The investigated group consisted of conservatively drug treated (n = 30) and patients with curative surgery (n = 25) with confirmed AE. The study was performed at an infectious disease outpatient department from April 2018 to October 2018. The conservatively drug treated patient group consisted of 15 men (50.0%) and 15 women (50.0%) with an average age of 55.7 ± 16.7 years (Median: 59). The surgery group consisted of nine men (36.0%) and 16 women (64.3%) with an average age of 53.3 ± 31.9 years (Median: 54). The physical quality of life of the conservatively drug treated patients did not show any significant differences to the surgical treated group (45.2 ± 11.4 vs. 47.6 ± 9.9; p = 0.4079). There was also no significant difference regarding the mental quality of life between the conservatively drug treated patients, and those treated with curative surgery (45.5 ± 10.6 vs. 47.3 ± 10.8; 0.5206). Nevertheless, there was a slight advantage in the physical and mental scores of the patients treated with surgery. Furthermore, for 13 of the 25 surgically treated patients, some aspects of the HRQoL improved significantly after surgery.The evaluation showed no statistically significant differences in HRQoL in patients with AE dependent on the applied treatment strategy (conservative drug versus curative surgical therapy).