We describe a case of human lagochilascariasis, with skull-base involvement and a chronic and relapsing course after treatment. This rare parasitic infection is usually manifested in the head and neck area, characterized by progressive granulomatous inflammation and the formation of abscesses. Transmission to humans most likely occurs by the consumption of undercooked meat of wild rodents. On the basis of literature studies, we propose the most likely life cycle of the parasite that involves wild feline and rodent species, with humans as accidental hosts. Even in endemic areas, it is very difficult to recognize the disease at an early stage. Progression will eventually lead to involvement of the (central) nervous system, as described in our case. Treatment is often difficult and involves resection and prolonged treatment with anthelmintic drugs. Recurrences are not uncommon and at present, long-term oral administration of ivermectin seems to be the most effective treatment.