Gastric cancer serves a major role in the global cancer burden, being the fourth most frequent cause of mortality among all types of cancer. Gastric squamous cell carcinoma (GSCC) is a rare histological variant accounting for 0.04-0.5% of all gastric cancer cases. Diagnostic work-up of GSCC is essential and involves multiple criteria: i) Tumour not located in the cardia, ii) no oesophageal extension of the tumour, and iii) no evidence of SCC in any other part of the body. Little is known about this rare variant in terms of pathogenesis, risk factors or evolution. Consequently, neither the European Society of Medical Oncology nor the National Comprehensive Cancer Network societies have published recommendations for GSCC. The aim of the present review is to provide an in-depth analysis of the current literature on this pathology, from pathophysiological hypothesis and clinical presentation to diagnostic work-up and treatment trends, in order to establish a possible management algorithm.