Primary hepatic yolk sac tumour (YST) is a very rare neoplasm. The authors present a case of a 20-year-old woman with fever and pain in the epigastrium, clinically resembling acute appendicitis along with high levels of serum alpha-fetoprotein. From laparoscopic appendectomy there was bioptically verified a diagnosis of YST. Consequently, there was discovered a tumor in the liver and the patient underwent right hemihepatectomy, hemicolectomy and ovariectomy, from which the YST was disclosed in the liver and in the coecum with spread into regional lymph nodes. While the gonadal, mediastinal and central nervous system origin of the YST was excluded, the diagnosis of the primary hepatic YST could have been established. The exact etiology of primary hepatic YST is still unclear. It has been suggested that these YSTs might originate from the germ cells that escaped during migration course from the yolk sac to the genital ridge during embryogenesis with subsequent malignant transformation or from persistent pluripotent embryonic cells in the liver, which escaped from the influence of differentiation during embryogenesis. This tumour is aggressive with ability to spread into distant sites, where it can lead to the first clinical presentation.