Introduction: West syndrome is a peculiar age dependent epilepsy syndrome, characterized by constellation of clustered spasms and hypsarrhythmia pattern on EEG. There is data from Indian studies which suggest male preponderance in children with West syndrome. The objective of the present study was to investigate this skewed gender ratio. Methods: We investigated this association via systematic review of published studies from India. We used a systematic review strategy as there is no national population based registry for West syndrome. We searched in PubMed, EMBASE, Scopus and Web of Science database for publications in English language from January 2001 to November 2013 using key terms: West Syndrome OR Infantile Spasms and India. Data on gender predominance and demographics were extracted from all the sources and incorporated in our study. Results: We identified four eligible published studies. A total of 391 children with West syndrome in these studies were assessed for gender ratio. Male preponderance was observed in 72.9% of study population. There is also significant interregional variation in gender ratio. Conclusion: Our study clearly demonstrated significant high male prevalence in children with West syndrome in India. There is a clear need of prospective multicenter study to confirm this observation and assess its implications.