Introduction: Ganglioneuromas (GN) are derived from neural crest cells associated with the autonomic ganglia, are an exceedingly rare finding in the appendix. GN are considered a benign lesion with an indolent course and a very low potential for malignant transformation. With no clear guidelines on whether surgical intervention is required for all GN, the difficulty of pre-operative diagnosis often leads to surgical resection. Case: An 85yo M was referred to a colorectal surgeon with an appendiceal nodule bulging into the caecum on colonoscopy, which had increased in size over 5 years. He was asymptomatic and had no significant medical history, including no history of genetic syndromes. A CT scan showed a normal calibre appendix, with no evidence of lymphadenopathy or distant disease. He proceeded to a laparoscopic caecetomy, with the pathology findings consistent with a benign mature ganglioneuroma. Discussion: GN in the appendix are rare, with only nine contemporary case reports. Most cases are asymptomatic, with acute appendicitis the most common presentation. Over half were associated with a genetic syndrome, mainly NF1. Pre-operative diagnosis is difficult, and imaging is non-specific, making pathological diagnosis post-surgery essential. While appendicectomy is typically sufficient for benign GNs, complications from surgery have led to some to suggest active surveillance for asymptomatic cases. Conclusion: Despite their benign and indolent nature, surgical resection remains the mainstay treatment for GNs especially when there is diagnostic uncertainty. The ultimate decision between surgical management and surveillance should be individualised, considering tumour characteristics, symptoms and surgical risk.