Myofibroma/myofibromatosis is a rare mesenchymal disorder that is part of a heterogeneous group of approximately 20 disorders that are classified primarily according to the proliferation of benign fibrous elements. These lesions can arise during a wide range of ages, with many occurring in the first decade of life, and they are slightly more common in males than females. The etiology of this disease is not well understood. Clinically, patients with myofibroma/myofibromatosis present with various signs, ranging from superficial, cutaneous, purplish macules to freely movable subcutaneous masses to deep-seated fixed lesions. The definitive diagnosis is made on histopathologic grounds. The destructive clinical behavior of myofibroma/myofibromatosis in the setting of insufficient pre- or perioperative diagnostic evaluations (e.g., a failure to perform fine-needle aspiration or frozen-section biopsy) may guide the clinician toward a radical surgical procedure rather than a simple excision.