Abrikossoff tumours or granular cell tumours are rare and usually benign tumours. The oesophagus is a rare location (0.001% of all tumours). The macroscopic appearance is typical (yellow, firm, well circumscribed submucosal neoplasm-like with reduced vascular patterns) but due to the rarity of this lesion, it can be mistaken with other similar lesions. This is the report of a case of a 47-year-old female with a granular cell tumour of the distal oesophagus. In this case, the Abrikossoff tumour is clinically, radiologically en histologically benign. According to the fact that there is low evidence in the literature for removing such Lesions in patient without symptoms, it was decided to be conservative. In this paper we discuss the case with review of the Literature with emphasis on the diagnosis, evaluation and management.