Rhabdomyoma is a rare benign tumor characterized histologically by striated skeletal muscle. Rhabdomyomas can be classified into adult and fetal variants. The majority of adult variants arise in the head and neck region and are derived from the skeletal muscles of the pharyngeal (branchial) arches. The majority of cases of fetal variant rhabdomyomas have been described in the postauricular region of young children and the vulvovaginal region in middle-aged women. We present a case of a 15-month-old infant with a circumferential rhabdomyoma of the cricopharyngeus muscle requiring excision of the esophageal inlet and reconstruction with a free microvascular jejunal interposition graft. This case represents the youngest reported case of adult variant rhabdomyoma. The clinical, radiographic, and histologic findings of this unusual case and a review of reported cases of rhabdomyoma in the pediatric population will be presented.