Oesophageal sarcomas are very rare while various histological types have occasionally been reported. Malignant Peripheral Nerve Sheath Tumour (MPNST) of the oesophagus is an exceedingly rare type of oesophageal sarcoma with only thirteen cases previously reported in the world literature. However, it should be included in the differential diagnosis of oesophageal neoplasias. Due to the small number of reported cases, the information about the biological behaviour of this entity is still insufficient. While MPNST is generally considered an aggressive type of tumour with high recurrence rates after surgical treatment and poor prognosis, previous reports of cases with oesophageal localization have recorded satisfactory outcomes overall even with less aggressive therapeutic approaches, although a long-term follow-up is lacking. Herein, we present the case of a 76-year-old female patient with oesophageal MPNST who presented with lymph node and distant metastases at the time of diagnosis, accounting for the second time only that this unusual presentation of this extremely uncommon disease has been reported. In our case, the course of disease was extremely aggressive which resulted in the second recorded death from this entity in the literature. The case presentation is followed by an extensive review of the world literature for the so far reported cases, aiming to highlight all relevant aspects such as demographics, clinical features, diagnostic assessment and findings, histological parameters, treatment and prognosis, and extract valuable previously unpublished conclusions for this rare entity.