Retrorectal primary mature cystic teratomas are extremely rare and can be challenging to diagnose and treat in adults. These lesions are frequently clinically unrecognized and misdiagnosed. We present a case of a 39-year-old male patient with mature cystic teratoma presenting with repeated episodes of meningitis. He presented to our emergency department with complaints of fever, headache and vomiting since 15 days. He was treated for initial diagnosis as tuberculosis meningitis by a specialist of infection disease with antibiotics after a CSF study which was culture negative. Since he had recurrent meningitis, radiological investigations revealed by suggested it to be a retrorectal mature cyst 20cm in diameter which may be related with the cerebrospinal fluid. He underwent a total cyst excision of the lesion and histopathology confirmed a mature cystic teratoma. We identified about 25 cases of recurrent meningitis associated with dermoid and epidermoid cysts in the Medline literature search. A high index of suspicion is an important factor in making an early diagnosis; rectal examination and radiologic evaluation are also valuable.