Malignant peripheral nerve sheath tumors are rare tumors, especially in the newborn period. Diagnosis is based on clinical findings, radiography, and fine needle biopsy or tissue sampling. Ideal management is controversial and extremely difficult. The survival rate is extremely low. We present a case of a newborn in which this tumor is diagnosed in the first few days of life as an ulcerating tumor imbedded in the entire forefoot with already metastatic spread to the lymph nodes and abdomen. Questions arose on the best treatment in postpartum life. Below the knee amputation was performed for nursing purposes, but eventually a no-intention-to treat policy was chosen by the multidisciplinary team and parents. Detection of soft tissue tumors in the prenatal period is difficult and not as easy as for other structural defects. Would prenatal detection have changed the course of life of this young infant? Will future opportunities for detection of soft tissue tumors occur? Diagnostic and therapeutic considerations are discussed alongside the present literature.