Colloid cysts are benign tumors located in the region around the foramen of Monro, or around the third ventricle with an annual incidence of 3 of 1,000,000. With a clinical progression of headache associated with hydrocephalus, brain herniation due to rapid enlargement of the colloid cyst may lead to death. A nineteen-year-old female was seen due to a three-month history of progressive headache, with associated signs of increased intracranial pressure and with a medical history of neurofibromatosis type 1, inherited from her mother. Neurologic findings revealed papilledema, and 6th cranial nerve palsy. By radiographic imaging with clinical correlation, patient was diagnosed with colloid cyst in the 3rd ventricle obstructing the foramen of monro. Surgical intervention was done, and patient improved without complications. Neurofibromatosis is a hereditary neurocutaneous syndrome in which the skin, nervous system, bones, endocrine glands, and sometimes other organs are the sites of a variety of congenital abnormalities, often taking the form of benign tumors. For this case an intracranial colloid cyst was noted. Colloid cyst in a patient all though rare with an incidence of 0.5 – 1% of all primary brain tumors is a strong and practical diagnosis, as it is seen as the most common mass in the third ventricle and in the foramen of monro, with a clinical correlation of obstruction of CSF flow resulting to hydrocephalus. No published incidence rate of neurofibromatosis type 1 patients with intracranial colloid cyst has been reported yet. Surgical resection is the treatment of choice for colloid cyst.