Objectives: To describe and analyse the epidemiological and clinical characteristics of imported human angiostrongyliasis in Europe. Methods: A systematic literature review of cases of human angiostrongyliasis in Europe was performed. Seven databases were searched. The epidemiological and clinical characteristics were extracted from included records and simple summary statistics were performed on extracted data. Results: Twenty-two cases reported between 1988 and 2019 were identified. They were mainly from French Polynesia, Southeast Asia, and the Caribbean Islands. The dominant suspected mode of transmission was ingestion of prawns, shrimp, or salad. For patients with data, 90% had a history of headache, often lasting, and half had paresthesia. Eighty-nine percent had eosinophilia, 93% had cerebrospinal fluid (CSF) eosinophilia, and 92% had elevated CSF protein. Central nervous system (CNS) imaging was normal in most cases. Two-thirds received albendazole or mebendazole treatment, although this is not currently recommended. Conclusions: We have increased previous numbers to 22 reported cases in total since 1988. Angiostrongyliasis should generally be suspected in patients with a lasting headache who have returned from Southeast Asia, China, the Caribbean Islands, Australia, or French Polynesia, as well as parts of North America and Tenerife, Spain, although one autochthonous case from mainland Europe has also been reported. A dietary history should focus on prawns, shrimp, and salad, whilst also including slugs and snails and other paratenic hosts where relevant. The clinical diagnosis is supported by the presence of blood eosinophilia, CSF eosinophilia, and elevated CSF protein. A definitive laboratory diagnosis should be sought, and CNS imaging should be used to support, not to rule out the diagnosis. The most up-to-date evidence should always be consulted before initiating treatment. Current recommendations include analgesics, corticosteroids, and periodic removal of CSF for symptom relief, while antihelminthic treatment is debated.