Background: Chordomas are rare slowly growing tumors that originate from remnants of the notochord. They have a malignant local behavior, causing symptoms due to bone infiltration and compression of neurovascular structures. Only a few cases of brain tumors associated with pathological laughter have been reported in the literature.
Case description: We report a case of a 42-year-old male patient with this atypical clinical presentation treated at our institution, and discuss the concerning literature.
Conclusion: Although being a very rare presentation of chordomas, pathological laughter is usually expected to improve after brain stem decompression.
Keywords: Chordoma; clivus; pathological laughter.