GIDID-1 was first described by Dallaire and Perreault in 1974. They reported five cases of intestinal atresia in three French Canadian families with common ancestors, and consanguinity was reported in two of the sibships. Intraluminal calcifications were present in radiologic imaging. GIDID-1 lesions are located in multiple levels of the gastrointestinal tract, from the stomach to the anus. Some patients present with Inflammatory Bowel Disease (IBD) signs and symptoms and may exhibit either mild or severe combined immunodeficiency. The first symptoms present in the early days or months of life, with an average onset at the age of 8.6 months. The digestive atresias predominantly occur in the small intestine followed by the colon. Histologic findings show the presence of apoptotic enterocolitis. Immunodeficiency is diagnosed in 95.4% of the cases and at an average age of 18.4 months. Three distinct phenotypic combinations have been identified: immunodeficiency and multiple intestinal atresia without enteropathy (ID/MIA), immunodeficiency and enteropathy without atresia (ID/E), immunodeficiency, multiple intestinal atresias and enteropathy (ID/MIA/E) which account for 21.8%, 34.5%, and 38.1% of the cases respectively.