Background <p>Primary spinal myoepithelioma is an exceedingly rare condition that frequently poses a diagnostic challenge due to its radiological similarity to other spinal tumors. Here, we report a rare case of primary cervical spinal myoepithelioma in an adolescent, emphasizing its diagnostic pitfalls and surgical management.</p> Case presentation <p>A 14-year-old male who presented with neck pain, left-hand numbness, and weakness, ultimately diagnosed with primary myoepithelioma of the cervical spine. The initial imaging studies, including magnetic resonance imaging (MRI) and computed tomography (CT), demonstrated a well-defined, expansile, dumbbell-shaped mass in the cervical vertebra, with no evidence of vertebral body destruction. The patient underwent gross total resection via a posterior midline approach with C3–C5 stabilization. Histopathological and immunohistochemical analyses supported a diagnosis consistent with myoepithelioma, although the immunophenotype was not entirely typical. Postoperatively, neurological symptoms improved significantly, and no recurrence was observed during 20 months of follow-up.</p> Conclusions <p>Primary spinal myoepithelioma should be considered in the differential diagnosis of dumbbell-shaped spinal lesions, even in pediatric patients. While the immunophenotype may be atypical (S-100 negative), comprehensive immunohistochemical profiling and gross total resection are essential for an accurate diagnosis and favorable clinical outcome. This case highlights the diagnostic challenges of rare spinal tumors and underscores the importance of integrating imaging, surgical, and pathological findings.</p>