Background: Tumor-like lesions at the craniovertebral junction mimic tumors in clinical presentation and imaging. Our study focuses on three common developmental pathologies—epidermoids, dermoids and neurenteric cysts. Methods: We conducted a retrospective analysis of a case series and a meta-analysis of 170 patients from 119 reports. Results: Neurenteric cysts predominated (81.2%). Anterior cysts were linked to neurenteric cysts, while posterior ones correlated with dermoid/epidermoid cysts (p < 0.001). Complications occurred in 27.2% of cases, with cranial nerve paresis being the most common. Most patients had excellent outcomes (75.2%) with low recurrence rates (12%). Dermoid cysts were more associated with anomalies (p < 0.001). Among 138 neurenteric cyst cases, 15 experienced recurrence, with predictors including ages 51–60 and over 70, subtotal resection, complications, and poor outcomes (p < 0.001). Cysts with total resection were significantly less likely to adhere to surrounding brain tissue (p < 0.001). CSF diversion was correlated with older age (p = 0.010) and various complications (p < 0.001). Age affected outcomes, and the hydrocephalus was linked to poor outcomes (p = 0.002). Conclusions: This meta-analysis underscores the importance of total resection in minimizing recurrence rates and emphasizes meticulous preoperative planning and imaging. Our results indicate that rim enhancement (p = 0.047) and poor outcome (p = 0.007) are significant factors associated with recurrence. Additionally, associated anomalies, as well as the patient’s age and overall health, significantly influence the surgical outcomes and the likelihood of recurrence.
