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Primary hepatic leiomyoma (PHL) is a rare entity, with very few cases reported in the literature. Even more rarely, until now practically undescribed, is the transformation of a hepatic leiomyoma into leiomyosarcoma with pancreatic metastases. Here, we report a single case of the progression of PHL in primary hepatic leiomyosarcoma, with clinical–surgical and histopathological features, and we conducted a review of the literature of related cases that can be found.