Aims: Juvenile nasopharyngeal angiofibromas (JNAs) are a tumour with an unknown pathogenesis that often present in an advanced stage. Many controversies exist regarding management of JNAs including the management of residual, recurrent or unresectable disease. This paper presents the results of a systematic review exploring the natural history of residual or recurrent disease and modern management options for this patient population. Methodology: A systematic review was undertaken from inception to September 2024 of PubMed, Embase, Scopus and Web of Science databases. Inclusion criteria included original peer-reviewed papers with data relating to the natural history of residual, recurrent or unresectable JNAs and management options with outcomes for these patients. Eighty-three studies were screened by two authors and thirty-two were included. Data was electronically collated and descriptive statistical analysis and qualitative review of the data was conducted using Microsoft Excel. Results: Residual or recurrent JNAs can grow at a rate of 2-9mm/year. However, many remain stable and some even involute. There are many treatment options, besides surgery, for residual or recurrent disease, especially if surgical resection will cause unacceptable morbidity in young patients. Treatment modalities include radiotherapy, gamma knife surgery and hormonal agents. In some cases residual/recurrent disease was simply monitored with MRI or CT and without further intervention disease remained stable or completely regressed and the patient remained asymptomatic over the follow-up period. There are also emerging novel imaging techniques that propose higher sensitivity and specificity for monitoring residual/recurrent JNAs. Conclusion: More treatment options are emerging that may improve surgical outcomes in patients with advanced JNAs and improve long-term outcomes for patients with residual or recurrent disease, while also minimising long-term morbidity in this young population.