In two cases of ectopic localization of Angiostrongylus costaricensis adult worms and eggs were seen in the liver, causing a granulomatous inflammatory reaction with dense eosinophilic infiltration and necrosis. In the first case, although antibodies for A. costaricensis had been demonstrated, a clinical diagnosis of visceral larva migrans was recorded; however, further examination of a liver biopsy revealed eggs of A. costaricensis. In the second case, severity of the intestinal pathology masked the presence of lesions and an adult A. costaricensis that were discovered in a retrospective study of the liver 13 years later. A close similarity between the visceral larva migrans syndrome and ectopic localization (liver) of A. costaricensis is established.