Superficial acral fibromyxoma, also known as digital fibromyxoma, is an uncommon benign tumor of the subungual or periungual region of the fingers and toes that was first characterized in 2001. The tumor is more common in men, with a male-to-female ratio of 2:1 and is typically seen in adults between the ages of 40 and 60. The great toe and the index finger are most commonly involved, and more than 50% of tumors involve the nail unit. Cases involving other digits, the palm, wrist, heel, sole, posterior thigh, and leg have also been described. A history of preceding trauma may be elicited, but its role in the pathogenesis remains controversial. The tumor is usually asymptomatic, but it may be tender. When asymptomatic, the diagnosis is usually delayed. There is evidence from immunohistochemistry and fluorescence in situ hybridization studies to suggest that Rb1 deficiency drives the development of superficial acral fibromyxoma.