The parasite Echinococcus multilocularis, also known as the fox tapeworm, causes alveolar echinococcosis in humans, one of the deadliest chronic parasitic diseases in the world, which the World Health Organization classifies among neglected diseases. It is exclusively distributed in the Northern Hemisphere, and endemic areas include the countries of Central Europe. The parasite's life cycle involves wild animals, most commonly red foxes in Europe, as the definitive hosts where the adult stage of the tapeworm develops, and voles as intermediate hosts where the metacestode stage develops. By ingesting food contaminated with parasite eggs, humans can accidentally become part of the parasite's life cycle. E. multilocularis shows a tropism for the liver, and as it develops in the liver, it clinically mimics a malignant tumor or cirrhosis of the liver. Correct diagnosis and treatment are challenging due to the nonspecific symptoms and signs of the disease, the inexperience of clinicians in distinguishing it from other conditions like cystic echinococcosis, and the pathogenesis of the disease itself. Therapeutic options include surgical treatment, endoscopic and percutaneous interventions, and antiparasitic drugs, but recurrence of the disease is possible due to the parasite's ability to metastasize. Since the 1990s, the prevalence of the parasite in Europe has significantly increased, primarily due to complex ecological processes underlying it. Despite being surrounded by countries where alveolar echinococcosis is an endemic disease, Croatia was considered a country without the presence of the parasite until 2015, when E. multilocularis was first recorded in red foxes, followed shortly by the first human infection case recorded in Vukovar in 2017. With the increase in infection incidence in the Bjelovar-Bilogora County, it has become a new hotspot in Croatia, suggesting the spread of the parasite from the east to the central part of the country. Data on the epidemiological situation related to E. multilocularis in Southeast Europe indicate the spread of the infection towards the south of the continent.