Background Lipochoristomas are benign fatty tumours with rare occurrence in internal acoustic canal and cerebellopontine angle (0.14%). Despite its benign nature and slow growth potential, it poses serious dilemma for neurotologists due to its intimate involvement with the auditory nerve. Case report We would like to present a case of distal intracanalicular lipochoristoma in a 60 year male with 5 years radiological follow up and a brief literature review of this rare entity. Conclusion Magnetic resonance imaging plays an important role in both diagnosing and surveillance of the disease. Due to limited surgical outcome, conservative surveillance is the best treatment till date.