Introduction: Intracranial dermoid cysts are benign, ectopic squamous epithelial cysts often compose of dermal structures like hair follicles, sweat glands as well as sebaceous glands. This lesions constitutes about 0.5% of all intracranial neoplasms. Thus, the occurrence of a dermoid cyst in the posterior fossa is very rare. We report a rare case of intracranial dermoid cyst in posterior cranial fossa. Case Presentation: Our first case was a 32 years old woman who presented with headaches and dizziness with no nausea, vomiting or fever. CT scan revealed a mass at occipital cistern consistent with a cystic lesion. MRI also revealed an irregular lesion in the posterior part of the medulla oblongata with enhanced edges signifying calcifications. We attained total resection of the tumor in a piece meal approach via surgery. Histopathology confirmed dermoid cyst. Two years follow-up revealed no recurrence of the lesion and no neurological deficits. Conclusion: We advocate that, the goal in surgical decision-making should be safe and total resection while monitoring the cranial nerves with electromyographic and auditory brainstem responses.