Sudden infant death syndrome (SIDS) is an irreparable loss for the parents and has life-long consequences for the grieved family Objective: This systematic review aims to compile all the researches conducted so far on the possible involvement of respiratory neuronal structures of brainstem in SIDS Methods: Studies were extracted by using MeSH words “Sudden infant death syndrome, neuropathology” on Pubmed database and also search through hand screening. Results: A total of 114 studies were identified which were later further screened for inclusion criteria such as SIDS cases, original articles and case reports and brain nuclei involved in SIDS, while review articles and editorials were excluded. After further screening 40 studies were shortlisted. All the data was analyzed through excel, yearwise, country wise comparison was also conducted. Maximum studies (24) were published from Italy, most publications were from 2010-2020 (27). Upon final screening according to the inclusion of neuronal brain nuclei studies in SIDS, 27 studies were finalized and used for analysis. Altogether there were 805 SIDS cases and 270 controls reported in these 27 studies. Alterations in the brain nuclei included arcuate nuclei, Dorsal raphe nuclei, preBötzinger complex, vagal nuclei, Kölliker-Fuse complex, Tractus solitarius, Parabrachial Kölliker-Fuse complex, Trigeminal nucleus, Parafacial/facial complex, Dentate gurus and others Conclusions: This study highlights the importance of further research in the underlying mechanisms of SIDS. It should be investigated at neuropathological as well as molecular level to understand the mechanisms. It may guide and help to identify the vulnerable infants and save their lives in future. Key words: Neuropathology, Sudden infant death syndrome, crib death, Cot death, brainstem, breathing