Leiomyosarcoma (LMS), a smooth muscle connective tissue tumor, is a rare form of cancer which accounts for 5–10% of soft tissue sarcomas. This type of cancer is highly unpredictable. LMS is a resistant type of cancer and can remain in the dormant state for long time. It can recur in the later stages of life. LMS has been reported in different animals including humans. A wide literature search was done. The PubMed database was used to search for journal articles on the occurrence of LMS in different organs from 1950 to 2016. LMS has been reported to be associated with different organs, including esophagus, stomach, intestine, anus and uterus. In this article, an attempt has been made to review the studies based on occurrence of LMS with respect to the organs affected and frequency of publications. Finding the organ-associated occurrence of LMS may be useful in assessing the overall risk and formulating future cancer preventive strategies.