Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is rare in 
patients under the age of 40 in the absence of metabolic or familial 
predisposition. A high incidence of involvement of the transverse ligament of 
the atlas in CPPD deposition disease was reported. However, involvement of the 
craniocervical junction is rarely symptomatic. We report a rare case in a young 
male with severe idiopathic CPPD crystal deposition disease, including crowned 
dens syndrome in the cervical spine.