Abstract Superficial acral fibromyxoma is a benign and rare tumor of the soft tissues. It usually manifests itself through a painless mass of slow growth that affects mainly males in the fifth decade of life. It usually affects the distal region, with a polypoid or dome-shaped appearance. The histological appearance is of a dermal mass without capsule, with spindle-shaped fibroblasts in a storiform or fasciculated pattern in the myxocollagenous stroma. The immunohistochemical evaluation of superficial acral fibromyxoma is usually positive for CD34 and CD99, with variable positivity for epithelial membrane antigen. The treatment consists of complete excision of the tumor mass. A review of the current literature on superficial acral fibromyxoma was performed, with an emphasis on the number of cases reported, location, diagnostic methods, histological characteristics, differential diagnoses and treatment. A total of 314 reported cases of superficial acral fibromyxoma with variable locations were found in the current literature, mainly in the toes (45.8%) and fingers (39.1%). It has a slightly superior incidence in men (61%) and enormous variability in the age range of occurrence. Superficial acral fibromyxoma is a single soft-tissue tumor that should enter the differential diagnosis of periungual and subungual acral lesions; the treatment consists of simple excision. More studies are needed to better understand this pathology, which was first described in 2001.