Achalasia has been well recognized as an esophageal motility disorder for more than three centuries. However, etiology and pathogenesis are still elusive and genetic, infectious, degenerative and autoimmune mechanisms have been proposed. The important role of interstitial cells of Cajal (ICC) in formation of achalasia has been determined recently. These cells are found in various organs and their physiological role is still not clear. Another pathology related with ICC is the gastrointestinal stromal tumors (GIST). GIST has a wider range of associated disorders when compared to achalasia. This manuscript is a case report, in which achalasia and gastric GIST coexisted. Today the question whether there is a relationship between the two pathologies remains unanswered. Since achalasia is a rarely seen disease, extra luminal abdominal pathologies may be overlooked in the course of routine examination and treatment plan. Knowing more about the association between these two diseases may be helpful during the preoperative assessments and postoperative follow-up of patients with achalasia.