Leiomyomas rarely occur in the hand. To our knowledge, there have been no reports of a leiomyoma of the hand mimicking a pearl ganglion in the English literature. We report such a case with a leiomyoma of the right third finger in a 59-year-old woman. The tumor was excised together with the underlying sheet of tissue. The pathology revealed that the tumor was linked to the underlying structure of a vascular wall by a stalk of tumor tissue. This report serves to remind clinicians to include leiomyoma in the differential diagnosis when encountering a 'ganglion-like lesion'. Also, this report demonstrates the link between a leiomyoma and its underlying origin.