Peripheral nerve sheath tumors, specifically neurofibromas and schwannoma variants, occur both sporadically and in the setting of hereditary conditions, including neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and familial/sporadic schwannoma [1]. It is a frequent and polymorphic genetic disorder. The severity is related to the complications. The degeneration of neurofibroma is a very rare complication of neurofibromatosis [1]. Neurofibromatosis type 1 is a multisystemic disorder that can affect several organs [2]. Sporadic gastrointestinal neurofibroma occurs considerably less often [3]. It may have a course of abdominal pain, discomfort, nausea, dyspepsia, and vomiting. Many of these tumors are age-related, occur at specific anatomic locations, and have unique imaging features [2,3]. In literature, many patients have a variety of organs affected because there is a high prevalence of multiple tumors occurring in the same patient [3]. A 5-year-old male patient was admitted to our pediatric surgery unit with the complaint of abdominal pain and distention. The family noticed the development of pigmented patches at birth on his skin and black dotted pigmentation along the right side of the neck (Figure 1 A, B, and C). Approximately 2 years ago, the family experienced his frequent gastrointestinal presentation with an unexplained right neck with a right upper eyelid swelling, evolution of the right orbit, and right testicular swelling (Figure 2 A, B, and C). The patient underwent a close clinical, radiological, laboratory, and histopathology workup (Figure 3 A, B, and C), (Figure 4 A, B), and (Figure 5 A, B, and C). All image results concluded neck and orbit plexiform neurofibromas with abdominal neurofibromas. We protocoled the management accordingly.