AbstractVisceral leishmaniasis is an important neglected parasitic disease that is generally caused byLeishmania infantum,Leishmania donovaniandLeishmania chagasi. However, several causative species of cutaneous leishmaniasis (CL) causes an interstitial form of leishmaniasis which known viscerotropic leishmaniasis. The aim of this paper is a systematic review of the cases of viscerotropic leishmaniasis to present the main causative agents, clinical manifestations, treatment and outcomes of the cases. An electronic search (any date to August 2017) without language restrictions was performed using Medline, PubMed, Scopus and Google Scholar. The searches identified 19 articles with total 30 case reports. Of them, old worldLeishmaniaspecies was reported from 23 (76.7%) cases, including 20 cases ofL. tropicaand three cases ofL. major; whereas new worldLeishmaniaspecies were reported in seven (23.4%) cases. The infection was more prevalent in male (24/30, 80%) than female (5/30, 16.7%) patients. Co-morbidity/co-infection was observed in 13 out of 30 cases (43.4%), which the most of them was HIV/AIDS (10 out of 13 cases, 76.9%). The results suggested that viscerotropic leishmaniasis should be more attended in the endemic countries of CL and in immunocompromised patients in order to exact discrimination from other endemic infectious diseases.