Cleidocranial dysplasia is a rare, autosomal dominant disease that is associated with clavicular absence or hypoplasia. Permanent pacemakers are most commonly implanted using percutaneous subclavian venous access. The clavicle is typically used as a bony landmark to guide venous access. Transvenous pacemaker implantation in the setting of clavicular hypoplasia, resection or other anomalies has not been described in literature. This is the first case report of a patient with clavicular absence undergoing transvenous permanent pacemaker implantation. This patient has a rare condition called cleidocranial dysplasia resulting in the congenital absence of his clavicles, along with other skeletal abnormalities. Cardiac anomalies are not associated with this disorder. This patient presented for permanent pacemaker placement in the setting of trifascicular block, symptomatic intermittent second-degree Mobitz type II atrioventricular block, and syncope. Using intra-procedural subclavian venography and intraprocedural Sonosite ultrasound imaging to identify vascular anatomy and surrounding anatomic landmarks, this patient underwent successful placement of a dual chamber transvenous pacemaker. Images from the intraprocedural venogram and the post-procedure chest x-ray illustrate the anatomy in this patient with congenital absence of the clavicles. This case has important implications in subclavian access and pacemaker placement in patients with clavicular abnormalities that may include absent, deformed, or resected clavicles.