Patient 1 (Pt1) suffered severe intestinal symptoms refractory to immunosuppressive medications and recurrent episodes of Guillain-Barre syndrome (GBS) besides infectious features. A literature review on the association of inborn errors of immunity (IEI) with nervous system pathologies suggests an array of central or peripheral nerve manifestations, including chronic inflammatory demyelinating polyneuropathy (mainly in autoimmune polyendocrinopathy-candidiasis-ectodermal-dystrophy syndrome), are linked to different IEI categories [[7]]. 10.1177/0883073817754176 9 Ozen A. CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease. Four patients developed lymphoma: diffuse large B cell lymphoma in three patients and EBV-positive Hodgkin lymphoma in one [[4]]. [Extracted from the article]