Introduction:Gastrointestinal basidiobolomycosis is an emerging infection, with fewer than 80 cases reported in the English literature.Case Presentation:Also, a few cases of gastrointestinal basidiobolomycosis, accompanied by liver involvement as part of a disseminated disease, have been reported.Conclusions:This is the first case report of an isolated liver involvement of this fungal infection in a 2-year-old girl, who presented with a liver mass resembling a hepatic abscess.