We report a 3-year-old boy who initially presented with abdominal pain and was subsequently found to have an esophageal perforation. The child did not respond to conservative management, and subsequent lymphadenopathy led to a lymph node biopsy demonstrating an anaplastic lymphoma kinase (ALK)+ anaplastic large cell lymphoma. Esophageal perforation and thickening is most commonly seen in children with a history of esophageal intervention or foreign body/caustic ingestion. Esophageal involvement in children with non-Hodgkin lymphoma (NHL) has not, to our knowledge, been reported in the literature. This case illustrates an unusual presentation of pediatric NHL.