INTRODUCTION:We present the case of a newborn with sacrococcygeal teratoma and complex gastroschisis, complicated with intestinal perforation. This is an unusual association, with only 1 reported case worldwide, and this is the first reported case that survived with a good outcome. CLINICAL CASE:The treatment was conducted in 4 stages: gastroschisis closure and enterostomy delaying the treatment of the atresia, teratoma resection, correction of the atresia using the Santulli technique and closure of the stoma. It took 6 months and 18 days to complete the whole treatment. The patient showed excellent progress with good growth and subsequent development. DISCUSSION:The treatment conducted in 4 stages made possible the resolution of each pathology, with proper assessment and surgical planning, making possible the patient's development and growth while minimizing the risks of complications.
