Abstract: We report the case of a 53-year-old man with inflammatory pseudotumor (IPT) of the liver and spleen. This concomitant association has rarely been reported. The patient presented with a hypoechoic mass in the liver and a clinical picture of recurrent sepsis; hematochemical exams and imaging data were nonspecific. Antibiotic therapy improved the clinical course, but did not resolve it definitively. After 50 days of therapy, as the hepatic mass decreased a similar lesion appeared in the spleen. The final diagnosis was made on splenectomy and an intra-operative biopsy of the residual liver lesion. The diagnostic problems encountered in this very rare association of IPT of the liver and spleen were similar to those for isolated IPT in the respective single organ sites. After 15 months of follow-up, the patient is in good health and no recurrence of symptoms or masses has been observed.