Anaplastic large cell lymphoma (ALCL), anaplastic lymphoma kinase (ALK)-positive, is uncommon in infancy. We present an unusual occurrence of infant ALCL, ALK-positive, associated with hemophagocytic syndrome. To the best of our knowledge, there have been no cases of infant ALCL, ALK-positive, described that have been associated with hemophagocytic syndrome. Particularly in this age group, primary hemophagocytic syndrome is also a consideration, which raises particular differential diagnostic considerations.