Abstract:  Infantile digital fibromatosis is a rare benign fibromatous tumor characterized by both its location on fingers and toes and its distinctive light microscopic appearance. However, treatment modalities are not yet standardized. Surgical excision had been preferred in the past but has the disadvantage of a high recurrence rate. More recently, isolated instances of spontaneous regression have been reported with a short follow-up. We report four children with infantile digital fibromatosis, who underwent spontaneous regression after clinical monitoring and long-term follow-up. We recommend regular follow-up in order to allow for intervention in case of functional impact on the affected extremities.