The Royal Australasian College of Surgeons (RACS) Te Rautaki Māori cites the need for more research dedicated to health equity in surgery for Māori. However, the gaps in research for Māori in surgery have not yet been highlighted. This review is the first in a series of reviews named Te Ara Pokanga that seeks to identify these gaps over all nine surgical specialties. The aim of this study was to assess neurosurgical disease incidence and perioperative outcomes for Māori at any point from referral through to the postoperative period. A systematic review of Māori neurosurgical disease and care for Māori in NZ was performed. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement assisted study selection and reporting. Kaupapa Māori (Māori-centred) research methodology and the Māori Framework were utilized to evaluate Māori research responsiveness. Nine studies were included in this review. All studies were retrospective cohort studies and only two studies had at least one Māori clinical or academic expert named on their research team. Therefore, only one study was deemed responsive to Māori. Studies assessing long-term outcomes from the management of neurosurgical disease for Māori and patient and whānau experiences of neurosurgical care are lacking. This study indicates the limited scope of research conducted for Māori in neurosurgery. The broader clinical implications of this review highlight the need for good quality research to investigate access to and long-term outcomes from the management of neurosurgical disease for Māori.