We cared for nine patients who were less than 21 years old at the time of diagnosis of follicular mucinosis. Four had clearing of the lesions, four did not, and one was lost to follow-up. Follicular mucinosis, even in childhood, may be persistent and be associated with lymphoma, which can result in death. Because there is no clinical pattern or single histopathologic criterion that correlates with the outcome, clinical follow-up and assessment of several histopathologic criteria must be relied on when evaluating this condition in children.