A review was conducted to identify the most common causative agents of anisakidosis, the methods used for identification of the causative agents, and to summarize the sources of infection, and patients’ demographics. A total of 762 cases (409 articles, inclusive of all languages) were found between 1965 and 2022. The age range was 7 months to 85 years old. Out of the 34 countries, Japan, Spain, and South Korea stood out with the highest number of published human cases of anisakidosis, respectively. This raises the question: Why are there few to no reports of anisakidosis cases in other countries, such as Indonesia and Vietnam, where seafood consumption is notably high? Other than the gastrointestinal tract, parasites were frequently found in internal organs such as liver, spleen, pancreas, lung, hiatal and epigastric hernia, and tonsils. There are also reports of the worm being excreted through the nose, rectum, and mouth. Symptoms included sore throat, tumor, bleeding, gastric/epigastric/abdominal/substernal/lower back/testicular pain, nausea, anorexia, vomiting, diarrhea, constipation, intestinal obstruction, intussusception, blood in feces, hematochezia, anemia, and respiratory arrest. These appeared either immediately or up to 2 months after consuming raw/undercooked seafood and lasting up to 10 years. Anisakidosis commonly mimicked symptoms of cancer, pancreatitis, type I/II Kounis syndrome, intussusception, Crohn’s disease, ovarian cysts, intestinal endometriosis, epigastralgia, gastritis, gastroesophageal reflux disease, hernia, intestinal obstruction, peritonitis, and appendicitis. In these cases, it was only after surgery that it was found these symptoms/conditions were caused by anisakids. A range of not only mainly marine but also freshwater fish/shellfish were reported as source of infection. There were several reports of infection with >1 nematode (up to >200), more than one species of anisakids in the same patient, and the presence of L4/adult nematodes. The severity of symptoms did not relate to the number of parasites. The number of anisakidosis cases is grossly underestimated globally. Using erroneous taxonomic terms, assumptions, and identifying the parasite asAnisakis(based solely on the Y-shaped lateral cord in crossed section of the parasite) are still common. The Y-shaped lateral cord is not unique toAnisakisspp. Acquiring a history of ingesting raw/undercooked fish/seafood can be a clue to the diagnosis of the condition. This review emphasizes the following key points: insufficient awareness of fish parasites among medical professionals, seafood handlers, and policy makers; limited availability of effective diagnostic methodologies; and inadequate clinical information for optimizing the management of anisakidosis in numerous regions worldwide.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00436-023-07881-9.