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Nevus lipomatosus cutaneous superficialis (NLCS) is a rare benign hamartomatous lesion characterized by ectopic mature adipocytes within the dermis. It most commonly affects the pelvic and gluteal regions, typically presenting as soft, skin-colored to yellowish papules or plaques that may gradually enlarge and coalesce over time. Although generally asymptomatic, untreated lesions can reach considerable size, leading to cosmetic deformity, functional impairment, and challenges in wound coverage. We present a case of a giant NLCS involving the craniocervical and thoracolumbar regions, an exceedingly uncommon distribution, with progressive enlargement since childhood. Clinical examination revealed extensive lobulated, cerebriform cutaneous masses spanning both regions. Diagnosis was confirmed through tissue biopsy demonstrating mature adipocytes dispersed among dermal collagen bundles, without extension into the subcutaneous tissue, consistent with NLCS. Given the lesion’s size and location, management required a staged, multidisciplinary strategy. Serial excisions were performed to gradually reduce tumor bulk while permitting wound adaptation. Large resultant defects were successfully reconstructed using split-thickness skin grafting. The patient recovered well, with satisfactory functional and aesthetic outcome and no evidence of recurrence at follow-up. To our knowledge, this represents the first documented case in the Philippines of a giant NLCS affecting both craniocervical and thoracolumbar regions. This report underscores the importance of recognizing atypical presentations of NLCS and highlights the value of staged excision combined with appropriate reconstructive techniques in managing extensive lesions.