BACKGROUND: Abrikossoff tumor is an uncommon neoplasia, benign in most of the cases, that affects soft tissues, skin, and oral mucosa. Between 1% and 2% of cases are malignant and, in these cases, outcome is usually fatal. Beetween 5% and 25% of patients have multiple lesions. CASE REPORT: A 52-year old Caucasian male was referred to the Plastic Surgery Department with a lesion in the tongue. A wide surgical excision was subsequently performed and the final pathologic diagnosis was Abrikossoff’s tumor. DISCUSSION: The pathogenesis of this tumor has long been subject of research and debate, and its origin has still to be clearly established. At the time, Abrikossoff proposed a myogenic origin, later studies supported a neural differentiation. According to the data in the literature the expression of S-100, that we found in our case, sustains the hypothesis that Abrikossoff tumor has origin in the Schwann’s cells. CONCLUSIONS: In our opinion, Abrikossoff tumor has origin in the Schwann’s cells. However familial cases, congenital cases, and multiple lesions are uncommon, and it is worth stressing that this tumor has a good prognosis but every patient with Abrikossoff tumor should undergo a complete physical examination to rule out the presence of multiple associated tumors and possible visceral involvement.