Background: Hirayama disease (HD) is characterized by progressive cervical myelopathy caused by repetive neck flexion leading to forward displacement of the posterior dural sack with compression and injury of the spinal cord. Typically, the C7-T1 myotomes become weak and atrophic, while sparing sensation. Here we present two Canadian cases of this rare entity. Methods: Two cases of HD are presented and literature reviewed, showing the diagnostic and therapeutic challenges of this disease. Results: Case 1 is a 17-year-old male professional singer and musician. He presented with bilateral progressive hand weakness, which was aggrevated while playing the violine. Cervical MRI showed increased T2-weighted signaling at C5-7, but a correct diagnosis could not be identified. Eventually, dynamic cervical MRI showed the compression and he underwent an anterior cervical discectomy and fusion (ACDF) at C5-C6 and C6-C7 without complications. Case 2 is a 19-year-old female with progressive right hand weakness. After numerous investigations, a dynamic cervical MRI diagnosed her with HD with classic findings and she underwent an ACDF at C6-C7 without complications. Conclusions: Hiryama’s disease is rare, but should be kept in mind when cervical cord signal changes cannot be explained by standard MRI. Dynamic MRI is imperative to correct diagnosis and anterior fusion shows good outcomes in its management.