Osteoid osteoma is a benign bony pathology. It presents either as a solitary lesion or as multiple lesions with a genetic predisposition. Reported more often in teenagers with thrice more common incidence among boys than in girls, it has a predilection for long bones of lower limbs. Less commonly arising from iliac crest or ribs; it is seen to be further rare to have originated from vertebrae or tarsal/carpal bones. Cranial osteomas are detected either incidentally on imaging or present as a bony hard swelling arising from the skull. Spinal intracanal osteomas are extremely rare to encounter in clinical practice. C ervical intracanal lesion in a case of hereditary multiple exostoses (HME ) presenting with myelopathy is further rare. Less than thirty such cases have been reported so far. We present here a rare case of HME in a 16-year-old boy with compressive myelopathy secondary to intracanal cervical osteoma at C4 Lamina and spinous process. He had a phenotypical expression of hereditary multiple osteomas with a strong family history of inheritance of trait among first-degree male relatives favoring genetic transmission of disease with variable penetrance. All reported cases, to date, are discussed in a tabulated form.