Ecchordosis physaliphora (EP) is a rare non-malignant mass that originates from remains of the notochord and is typically asymptomatic. Symptomatic cases are extremely rare, and the majority are managed by surgical resection. This case study reports a 42-year-old male who presented with sudden onset of painless horizontal diplopia which examination revealed to be caused by a sixth cranial nerve palsy. Magnetic resonance imagining (MRI) revealed a non-enhanced retroclival mass (EP) with increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. He was treated with methylprednisolone and completely recovered in four weeks, remaining symptom free at subsequent follow-up. In conclusion, EP is mainly an asymptomatic lesion and its presence with a sixth nerve palsy is not necessarily a causation. The authors discuss that conservative management should be attempted before surgery in all cases, due to the fact that symptoms can resolve spontaneously and EP could be an incidental finding. The role of steroids in the management of EP is uncertain at this time and warrants further investigation.